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Article: Apparent mineralocorticoid excess - update.

Nimkarn, Saroj

Advances in experimental medicine and biology

2011 Volume 707, Page(s) 47–48

MeSH term(s)	Humans ; Hypertension/blood ; Hypertension/etiology ; Hypertension/urine ; Mineralocorticoids/metabolism
Chemical Substances	Mineralocorticoids
Language	English
Publishing date	2011
Publishing country	United States
Document type	Journal Article
ISSN	2214-8019 ; 0065-2598
ISSN (online)	2214-8019
ISSN	0065-2598
DOI	10.1007/978-1-4419-8002-1_10
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Article ; Online: Congenital adrenal hyperplasia in adults.

Lin-Su, Karen / Nimkarn, Saroj / New, Maria I

Expert review of endocrinology & metabolism

2019 Volume 4, Issue 1, Page(s) 67–77

Abstract: Hyperandrogenism in congenital adrenal hyperplasia (CAH) results from overstimulation of adrenocorticotropic-driven androgen production in the adrenal cortex due to lack of cortisol feedback. The classical form is characterized by more-severe symptoms of ...

Abstract	Hyperandrogenism in congenital adrenal hyperplasia (CAH) results from overstimulation of adrenocorticotropic-driven androgen production in the adrenal cortex due to lack of cortisol feedback. The classical form is characterized by more-severe symptoms of hyperandrogenism, including virilization of the female genitalia. The milder nonclassical form presents with postnatal symptoms of hyperandrogenism. Presenting symptoms in adulthood may include acne, male-pattern alopecia, hirsutism, irregular menses/amenorrhea or infertility. The goal of therapy in CAH is to both correct the deficiency in cortisol secretion and suppress androgen overproduction. Glucocorticoid replacement has been the mainstay of treatment for CAH but new treatment strategies continue to be developed and studied.
Language	English
Publishing date	2019-03-21
Publishing country	England
Document type	Journal Article
ISSN	1744-8417
ISSN (online)	1744-8417
DOI	10.1586/17446651.4.1.67
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Article ; Online: Perioperative stress dose steroid management of children with classical congenital adrenal hyperplasia: Too much or too little?

Yau, Mabel / Jacob, Marianne / Orton, Sarah / Howell, Joy D / Lekarev, Oksana / Vogiatzi, Maria G / Poppas, Dix / Nimkarn, Saroj / Lin-Su, Karen

Journal of pediatric urology

2021 Volume 17, Issue 5, Page(s) 654.e1–654.e6

Abstract: Background: Children with congenital adrenal hyperplasia (CAH) are at risk for adrenal crises in the perioperative period and require higher doses of glucocorticoids. However, there are no specific protocols detailing the appropriate stress dosing ... ...

Abstract	Background: Children with congenital adrenal hyperplasia (CAH) are at risk for adrenal crises in the perioperative period and require higher doses of glucocorticoids. However, there are no specific protocols detailing the appropriate stress dosing required for children with CAH undergoing surgery with anesthesia. Objective: To evaluate CAH patients using our current hydrocortisone stress dose surgical protocol. We hypothesized that current clinical protocols may overestimate the endogenous response to perioperative stress. Study design: 14 children with CAH scheduled to have genital surgery and a control group of 10 unaffected children scheduled to have cardiac or urologic surgery (of a similar duration) were evaluated in a prospective observational study. Urinary free cortisol (UFC) and urinary 17-hydroxycorticosteroids (17-OHCS) per body surface area were measured in the postoperative period. Results: UFC levels were significantly higher in CAH patients (115.8 ± 24.6 nmol/m Conclusion: In the immediate postoperative period, urinary cortisol and its metabolites are significantly higher in pediatric CAH patients receiving stress dose corticosteroids compared to controls. Results suggest that the amount of hydrocortisone given during our stress dose protocol may be higher than physiologic needs. Future dynamic studies are needed to determine appropriate perioperative and postoperative cortisol requirements in pediatric CAH patients in order to develop optimal stress dose regimens.
MeSH term(s)	Acute Disease ; Adrenal Hyperplasia, Congenital/drug therapy ; Child ; Glucocorticoids ; Humans ; Hydrocortisone ; Prospective Studies
Chemical Substances	Glucocorticoids ; Hydrocortisone (WI4X0X7BPJ)
Language	English
Publishing date	2021-07-02
Publishing country	England
Document type	Journal Article ; Observational Study
ZDB-ID	2237683-5
ISSN	1873-4898 ; 1477-5131
ISSN (online)	1873-4898
ISSN	1477-5131
DOI	10.1016/j.jpurol.2021.06.027
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Article ; Online: Effect of ethosuximide on cortisol metabolism in the treatment of congenital adrenal hyperplasia.

Yau, Mabel / Rao, Niva / Nimkarn, Saroj / Vogiatzi, Maria

Journal of pediatric endocrinology & metabolism : JPEM

2014 Volume 27, Issue 5-6, Page(s) 549–554

Abstract: Background: Antiepileptics may affect cortisol metabolism through CYP3A4. There is little known about ethosuximide.: Clinical case: Our patient is a 12-year-old girl with salt-wasting congenital adrenal hyperplasia (CAH) owing to 21 hydroxylase ... ...

Abstract	Background: Antiepileptics may affect cortisol metabolism through CYP3A4. There is little known about ethosuximide. Clinical case: Our patient is a 12-year-old girl with salt-wasting congenital adrenal hyperplasia (CAH) owing to 21 hydroxylase deficiency. A standard treatment regimen was initiated with satisfactory results until the age of 6 years, when she developed absence seizures treated with ethosuximide. She received such therapy until the age of 12 years, at which point ethosuximide was discontinued. During ethosuximide administration, she experienced worsening control of CAH disease activity that responded to progressive increases in hydrocortisone dose up to 28 mg/m2 per day. Despite high doses of hydrocortisone, she suffered no cushingoid symptoms. Her requirements for high glucocorticoid replacement doses resolved shortly after ethosuximide was discontinued. We provide data over 6 years demonstrating a correlation between adrenal hormone secretion, cortisol requirements and ethosuximide dose. Conclusion: This is the first case demonstrating an interaction between ethosuximide and hydrocortisone clearance in the treatment of salt-wasting CAH.
MeSH term(s)	Adrenal Hyperplasia, Congenital/drug therapy ; Anti-Inflammatory Agents/metabolism ; Anti-Inflammatory Agents/therapeutic use ; Anticonvulsants/adverse effects ; Child ; Drug Interactions ; Epilepsy, Absence/complications ; Epilepsy, Absence/drug therapy ; Epilepsy, Tonic-Clonic/complications ; Epilepsy, Tonic-Clonic/drug therapy ; Ethosuximide/adverse effects ; Female ; Humans ; Hydrocortisone/metabolism ; Hydrocortisone/therapeutic use
Chemical Substances	Anti-Inflammatory Agents ; Anticonvulsants ; Ethosuximide (5SEH9X1D1D) ; Hydrocortisone (WI4X0X7BPJ)
Language	English
Publishing date	2014-05
Publishing country	Germany
Document type	Case Reports ; Journal Article
ZDB-ID	1231070-0
ISSN	2191-0251 ; 0334-018X
ISSN (online)	2191-0251
ISSN	0334-018X
DOI	10.1515/jpem-2013-0147
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Zs.A 2258: Show issues

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Article ; Online: Congenital adrenal hyperplasia due to 21-hydroxylase deficiency: A paradigm for prenatal diagnosis and treatment.

Nimkarn, Saroj / New, Maria I

Annals of the New York Academy of Sciences

2010 Volume 1192, Page(s) 5–11

Abstract: 21-Hydroxylase deficiency is the most common cause of congenital adrenal hyperplasia (CAH), an inherited disorder of steroidogenesis. In its severe form, CAH causes genital ambiguity in females. Molecular genetic analysis of fetal DNA obtained by ... ...

Abstract	21-Hydroxylase deficiency is the most common cause of congenital adrenal hyperplasia (CAH), an inherited disorder of steroidogenesis. In its severe form, CAH causes genital ambiguity in females. Molecular genetic analysis of fetal DNA obtained by amniocentesis or chorionic villus sampling is used to diagnose steroid 21-OHD deficiency in utero. Large ongoing studies show that appropriate prenatal treatment of pregnant mothers with dexamethasone is effective and safe for both the fetus and the mother. It reduces ambiguous genitalia in the female affected fetus and thus avoids unnecessary genitoplasty in the newborn female.
MeSH term(s)	Adrenal Hyperplasia, Congenital/diagnosis ; Adrenal Hyperplasia, Congenital/genetics ; Adrenal Hyperplasia, Congenital/therapy ; Animals ; DNA Mutational Analysis ; Female ; Humans ; Models, Biological ; Pregnancy ; Prenatal Care/methods ; Prenatal Diagnosis/methods ; Steroid 21-Hydroxylase/genetics
Chemical Substances	Steroid 21-Hydroxylase (EC 1.14.14.16)
Language	English
Publishing date	2010-03
Publishing country	United States
Document type	Journal Article ; Review
ZDB-ID	211003-9
ISSN	1749-6632 ; 0077-8923
ISSN (online)	1749-6632
ISSN	0077-8923
DOI	10.1111/j.1749-6632.2009.05225.x
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Se 110: Show issues

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Article: Prenatal diagnosis and treatment of congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

Nimkarn, Saroj / New, Maria I

Molecular and cellular endocrinology

2009 Volume 300, Issue 1-2, Page(s) 192–196

Abstract: Congenital adrenal hyperplasia (CAH) applies to a group of inherited disorders caused by an enzyme deficiency in steroid biosynthesis. The most common form of CAH is 21-hydroxylase deficiency (21-OHD), which in its severe form can cause genital ambiguity ...

Abstract	Congenital adrenal hyperplasia (CAH) applies to a group of inherited disorders caused by an enzyme deficiency in steroid biosynthesis. The most common form of CAH is 21-hydroxylase deficiency (21-OHD), which in its severe form can cause genital ambiguity in females. Affected females experience virilization both physically and psychologically. Steroid 21-OHD can be diagnosed in utero through molecular genetic analysis of fetal DNA. Appropriate prenatal treatment by dexamethasone administration to the at-risk pregnant mother is effective in reducing genital virilization in the fetus, thus avoiding unnecessary genitoplasty in affected females. Current data from large human studies show that prenatal diagnosis and treatment are safe in the short term for both the fetus and the mother. Preliminary data from long-term studies support these results.
MeSH term(s)	Adrenal Hyperplasia, Congenital/diagnosis ; Adrenal Hyperplasia, Congenital/enzymology ; Adrenal Hyperplasia, Congenital/therapy ; Androgens/metabolism ; Female ; Fetus/physiology ; Gestational Age ; Humans ; Pregnancy ; Prenatal Diagnosis/methods ; Sex Differentiation ; Steroid 21-Hydroxylase/genetics ; Steroid 21-Hydroxylase/metabolism ; Treatment Outcome
Chemical Substances	Androgens ; Steroid 21-Hydroxylase (EC 1.14.14.16)
Language	English
Publishing date	2009-03-05
Publishing country	Ireland
Document type	Journal Article ; Review
ZDB-ID	187438-x
ISSN	1872-8057 ; 0303-7207
ISSN (online)	1872-8057
ISSN	0303-7207
DOI	10.1016/j.mce.2008.11.027
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Zs.A 1127: Show issues

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Article ; Online: Vaginal bleeding leading to incidental diagnosis of ovarian torsion in an infant with 21 hydroxylase deficiency congenital adrenal hyperplasia.

Choudhary, Abha / Spigland, Nitsana / Poppas, Dix / Kovanlikaya, Arzu / Nimkarn, Saroj

The Journal of pediatrics

2013 Volume 162, Issue 2, Page(s) 432–432.e1

MeSH term(s)	Adrenal Hyperplasia, Congenital/complications ; Female ; Humans ; Incidental Findings ; Infant ; Ovarian Diseases/complications ; Ovarian Diseases/diagnosis ; Torsion Abnormality/complications ; Torsion Abnormality/diagnosis ; Uterine Hemorrhage/etiology
Language	English
Publishing date	2013-02
Publishing country	United States
Document type	Case Reports ; Journal Article
ZDB-ID	3102-1
ISSN	1097-6833 ; 0022-3476
ISSN (online)	1097-6833
ISSN	0022-3476
DOI	10.1016/j.jpeds.2012.08.026
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Article: Steroid 11beta- hydroxylase deficiency congenital adrenal hyperplasia.

Nimkarn, Saroj / New, Maria I

Trends in endocrinology and metabolism: TEM

2008 Volume 19, Issue 3, Page(s) 96–99

Abstract: Congenital adrenal hyperplasia due to steroid 11beta-hydroxylase deficiency is a genetic disorder of steroidogenesis, transmitted as an autosomal recessive trait. It is associated with low renin hypertension, hypokalemia, hyperandrogenemia and genital ... ...

Abstract	Congenital adrenal hyperplasia due to steroid 11beta-hydroxylase deficiency is a genetic disorder of steroidogenesis, transmitted as an autosomal recessive trait. It is associated with low renin hypertension, hypokalemia, hyperandrogenemia and genital ambiguity in affected females. Mutations in the CYP11B1 gene, causing 11beta-hydroxylase deficiency in the zona fasciculata in the adrenal cortex, have been identified. The indicators of congenital adrenal hyperplasia caused by 11beta-hydroxylase deficiency, include increased serum concentrations of desoxycorticosterone, 11 deoxycortisol and delta4-androstenedione, and suppressed plasma renin concentrations. The disorder is treated by administration of glucocorticoids.
MeSH term(s)	Adrenal Cortex/metabolism ; Adrenal Hyperplasia, Congenital/complications ; Adrenal Hyperplasia, Congenital/genetics ; Adrenal Hyperplasia, Congenital/metabolism ; Glucocorticoids/therapeutic use ; Humans ; Hypertension/etiology ; Mutation ; Steroid 11-beta-Hydroxylase/genetics ; Steroid 11-beta-Hydroxylase/metabolism
Chemical Substances	Glucocorticoids ; Steroid 11-beta-Hydroxylase (EC 1.14.15.4)
Language	English
Publishing date	2008-04
Publishing country	United States
Document type	Journal Article ; Review
ZDB-ID	1042384-9
ISSN	1879-3061 ; 1043-2760
ISSN (online)	1879-3061
ISSN	1043-2760
DOI	10.1016/j.tem.2008.01.002
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Zs.A 2999: Show issues

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Article: Prenatal diagnosis and treatment of congenital adrenal hyperplasia.

Nimkarn, Saroj / New, Maria I

Hormone research

2007 Volume 67, Issue 2, Page(s) 53–60

Abstract: Congenital adrenal hyperplasia is a group of inherited disorders caused by an enzyme deficiency in steroid biosynthesis. The most common form of congenital adrenal hyperplasia is 21-hydroxylase deficiency, which in its severe form can cause genital ... ...

Abstract	Congenital adrenal hyperplasia is a group of inherited disorders caused by an enzyme deficiency in steroid biosynthesis. The most common form of congenital adrenal hyperplasia is 21-hydroxylase deficiency, which in its severe form can cause genital ambiguity in females. Steroid 21-hydroxylase deficiency can be diagnosed in utero through molecular genetic analysis of fetal DNA. Prenatal treatment successfully reduces genital ambiguity, and the subsequent problems of sex misassignment and gender confusion. Data from current studies show that prenatal diagnosis and treatment are safe for the mother and the fetus. The evidence also suggests that it is safe over the long term, but all subjects exposed to dexamethasone treatment during embryonic and fetal life should have their physical, cognitive and emotional developments recorded.
MeSH term(s)	Adrenal Hyperplasia, Congenital/diagnosis ; Adrenal Hyperplasia, Congenital/drug therapy ; Algorithms ; Dexamethasone/adverse effects ; Dexamethasone/therapeutic use ; Female ; Fetal Diseases/diagnosis ; Fetal Diseases/drug therapy ; Humans ; Pregnancy ; Prenatal Diagnosis ; Prenatal Exposure Delayed Effects ; Steroid 21-Hydroxylase/genetics ; Steroid 21-Hydroxylase/metabolism
Chemical Substances	Dexamethasone (7S5I7G3JQL) ; Steroid 21-Hydroxylase (EC 1.14.14.16)
Language	English
Publishing date	2007
Publishing country	Switzerland
Document type	Journal Article ; Research Support, N.I.H., Extramural ; Review
ZDB-ID	124442-5
ISSN	1423-0046 ; 0301-0163
ISSN (online)	1423-0046
ISSN	0301-0163
DOI	10.1159/000096353
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Article ; Online: Steroid 21 hydroxylase deficiency congenital adrenal hyperplasia.

Nimkarn, Saroj / Lin-Su, Karen / New, Maria I

Pediatric clinics of North America

2011 Volume 58, Issue 5, Page(s) 1281–300, xii

Abstract: Steroid 21 hydroxylase deficiency is the most common form of congenital adrenal hyperplasia (CAH). The severity of this disorder depends on the extent of impaired enzymatic activity, which is caused by various mutations of the 21 hydroxylase gene. This ... ...

Abstract	Steroid 21 hydroxylase deficiency is the most common form of congenital adrenal hyperplasia (CAH). The severity of this disorder depends on the extent of impaired enzymatic activity, which is caused by various mutations of the 21 hydroxylase gene. This article reviews adrenal steroidogenesis and the pathophysiology of 21 hydroxylase deficiency. The three forms of CAH are then discussed in terms of clinical presentation, diagnosis and treatment, and genetic basis. Prenatal diagnosis and treatment are also reviewed. The goal of therapy is to correct the deficiency in cortisol secretion and suppress androgen overproduction. Glucocorticoid replacement has been the mainstay of treatment for CAH, but new treatment strategies continue to be developed and studied.
MeSH term(s)	Adrenal Cortex Hormones/therapeutic use ; Adrenal Hyperplasia, Congenital/diagnosis ; Adrenal Hyperplasia, Congenital/metabolism ; Adrenal Hyperplasia, Congenital/therapy ; Bone Density ; Female ; Hormone Replacement Therapy ; Humans ; Male ; Molecular Diagnostic Techniques ; Pregnancy ; Pregnancy Complications/therapy ; Reconstructive Surgical Procedures ; Steroid 21-Hydroxylase/metabolism
Chemical Substances	Adrenal Cortex Hormones ; Steroid 21-Hydroxylase (EC 1.14.14.16)
Language	English
Publishing date	2011-10
Publishing country	United States
Document type	Journal Article ; Review
ZDB-ID	215711-1
ISSN	1557-8240 ; 0031-3955
ISSN (online)	1557-8240
ISSN	0031-3955
DOI	10.1016/j.pcl.2011.07.012
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