Management Approaches to Congenital Adrenal Hyperplasia in Adolescents and Adults; Latest Therapeutic Developments

Bahtiyar, Gül; Sacerdote, Alan

doi:10.5772/26350

Cited by 8 publications

(9 citation statements)

References 76 publications

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“…In 2006 we reported additional endocrine disrupter effects of two classes of drugs used in antiretroviral therapy that were already known to cause insulin resistance, protease inhibitors and nucleoside analogues-the induction/unmasking of adrenal hyperplasia [15].…”

Section: Recognition Of Insulin Resistance In Cahmentioning

confidence: 99%

Treatment of Congenital Adrenal Hyperplasia by ReducingInsulin Resistance and Cysticercosis Induced Polycystic Ovarian Syndrome

Sacerdote¹,

Bahtiyar²

2015

Contemporary Gynecologic Practice

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Among the most common causes of female infertility, anovulation, menstrual irregularity, hirsutism, acne, and alopecia are congenital adrenal hyperplasia (CAH) and polycystic ovarian syndrome (PCOS). These two conditions resemble one another quite a bit, especially the nonclassic forms of CAH (NCAH). Another common cause of such problems is hyperprolactinemia, which results in increased androgen synthesis by both the ovaries and the adrenal cortex, while suppressing gonadotrophin-releasing hormone, gonadotrophin, and estrogen synthesis. Hyperprolactinemia, in turn, may be caused by primary hypothyroidism, prolactinomas, stalk effects of other pituitary and hypothalamic neoplasia, as well as a host of prescription and recreational drugs; it may also be idiopathic. Other, less frequently encountered causes of these problems include Cushing's syndrome and virilizing tumors (ovarian, adrenal, or ectopic). A growing worldwide problem in this sphere is androgen doping to improve athletic performance. Additional causes of menstrual irregularity include uterine leiomyomata, puberty, perimenopause, chronic illnesses eg. poorly controlled diabetes mellitus and sickle cell disease, elite athletics and dancing, eating disorders, endometriosis, and Asherman's syndrome. Infertility may also be caused by stress, tubal factors, Asherman's syndrome, immune response to spermatozoa, luteal phase inadequacy, and male factors. In this chapter we shall focus on 3 novel concepts: • The treatment of the congenital adrenal hyperplasias and the acquired/unmasked adrenal hyperplasias by interventions which reduce insulin resistance.

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Section: Recognition Of Insulin Resistance In Cahmentioning

confidence: 99%

Treatment of Congenital Adrenal Hyperplasia by ReducingInsulin Resistance and Cysticercosis Induced Polycystic Ovarian Syndrome

Sacerdote¹,

Bahtiyar²

2015

Contemporary Gynecologic Practice

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“…Although glucocorticoid treatment (sometimes combined with fludrocortisone) continues to be the mainstay of conventional treatment of CAH and has been very helpful and lifesaving in that capacity, its use, even with recent refinements, continues to be plagued with issues regarding over-and under-treatment, growth inhibition, hypertension, obesity, low bone density, testicular adrenal rest tumors, and diabetes [58][59][60]. Our group has been focusing on insulin sensitizing interventions to reduce IR, which is a consistent feature of CAH, as it is with PCOS [61][62][63][64][65].…”

Section: Discussionmentioning

confidence: 99%

Effect of Vitamin D in a Patient With Classical Adrenal Hyperplasia due to 11-Hydroxylase Deficiency

Thomas

2013

J Med Cases

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Both classical and non-classical CAH, like PCOS, are characterized by insulin resistance (IR) and are also ameliorated by measures which reduce IR. Vitamin D supplementation reduces IR and ameliorates PCOS in Vitamin D deficient/insufficient (VDDI) patientsraising the question: would Vitamin D replacement also ameliorate CAH? Our patient is a 47 year old man with Type 2 diabetes (Type 2 DM) and hypertension hospitalized for cellulitis, abscess, osteomyelitis, and gangrene of the left foot. He was not taking any known insulin sensitizers, and denied any history of acne or hyperpigmentation. The patient has no progeny and never had intercourse due to issues concerning his phallus size. He had hypospadias and micropenis at birth. The former was surgically corrected in childhood. His BMI = 36.45 kg/m 2 with a central fat distribution. On physical examination there was no acanthosis nigricans or hyperpigmentation. He had a stage IVa Hamilton male pattern alopecia that started during his early 20s. He had micropenis (or clitoromegaly), measuring 1.5 -2cm when flaccid; scrotum (or scrotalized labia majora) was normal in size. Testes is measured 3.4 cm in their long diameter and were firm. On 12-4-2012 his serum 11-deoxycortisol by liquid chromatography tandem mass spectrometry (LC MS/MS) was 2024 ng/dL ( ≤ 76) while his serum 25-OH-vitamin D by LC MS/ MS was 12 ng/mL( ≥ 30), and his 1,25 (OH)2-Vitamin D3 by LC MS/MS was ≤ 8 pg/mL (18 -72). Supplementation was begun with ergocalciferol 50,000 units orally weekly. Nine days later his serum 11-deoxycortisol fell to 425 ng/dL, while serum 25-OH-Vitamin D rose to 23 ng/mL, and serum 1,25-(OH)2-Vitamin D3 remained ≤8 pg/mL. Seventeen days later his serum 11-deoxycortisol was 194 ng/dL, while his serum 25-OH-Vitamin D was 27 ng/mL, and his 1,25-(OH)2-vitamin D3 remained ≤ 8 pg/mL. 28 days after starting supplementation with ergocalciferol the 11-deoxycortisol was < 20ng/dL. The laboratory lost the corresponding sample for Vitamin D metabolites. His lymphocyte karyotype was XY. He refused any biopsies, which might have disclosed mosaicism.Vitamin D might be a means of treating classical 11-hydroxylasedeficiency in patients with a VDDI co-morbidity and suggest that it may work by reducing IR.

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“…Although glucocorticoid replacement has historically been the main method of treatment, our group has focused on using insulin sensitisers to improve insulin sensitivity 26––28…”

Section: Discussionmentioning

confidence: 99%

Roux-en-Y gastric bypass in the treatment of non-classic congenital adrenal hyperplasia due to 11-hydroxylase deficiency

et al. 2013

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Non-classic adrenal hyperplasia (NCAH) has been associated with insulin resistance (IR). Therapies such as metformin, thiazolidinediones and lifestyle alterations improve IR and also ameliorate the biochemical and clinical abnormalities of NCAH, much as they do in polycystic ovarian syndrome (PCOS). More recently, bariatric surgery, such as Roux-en-Y gastric bypass (RYGBP), has also been associated with improvement in IR and amelioration of PCOS and may, therefore, be beneficial in NCAH. We report a case of a 39-year-old, deaf-mute, obese woman with NCAH due to 11-hydroxylase deficiency who underwent RYGBP followed by improvement of NCAH manifestations. She was initially treated with metformin and pioglitazone, which lowered serum 11-deoxycortisol from 198 ng/dl (<51) to 26 ng/dl. Five weeks after undergoing RYGBP her body mass index fell from 44.18 kg/m2to 39.54 kg/m2and, despite not taking metformin or pioglitazone, serum 11-deoxycortisol remained normal at <40 ng/dl. Concurrently and subsequently, her NCAH symptoms, for example, alopecia, hirsutism and irregular menses normalised as well. We conclude that RYGBP, like other interventions that reduce IR, may be another way of treating non-classic 11-hydroxylase deficiency in selected patients.

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Management Approaches to Congenital Adrenal Hyperplasia in Adolescents and Adults; Latest Therapeutic Developments

Cited by 8 publications

References 76 publications

Treatment of Congenital Adrenal Hyperplasia by ReducingInsulin Resistance and Cysticercosis Induced Polycystic Ovarian Syndrome

Treatment of Congenital Adrenal Hyperplasia by ReducingInsulin Resistance and Cysticercosis Induced Polycystic Ovarian Syndrome

Effect of Vitamin D in a Patient With Classical Adrenal Hyperplasia due to 11-Hydroxylase Deficiency

Roux-en-Y gastric bypass in the treatment of non-classic congenital adrenal hyperplasia due to 11-hydroxylase deficiency

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