Corticotropin-Independent Cushing Syndrome in a Child with an Ovarian Tumor Misdiagnosed as Nonclassic Congenital Adrenal Hyperplasia

2008 ◽  
Vol 14 (7) ◽  
pp. 875-879 ◽  
Author(s):  
Punkaj Gupta ◽  
Sunali Goyal ◽  
Luis Gonzalez-Mendoza ◽  
Natan Noviski ◽  
Marko Vezmar ◽  
...  
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Ovarian Tumor ◽  
Cushing Syndrome ◽  
Adrenal Hyperplasia

scholarly journals Late-onset Congenital Adrenal Hyperplasia with Cushing Syndrome

2014 ◽  
Vol 53 (17) ◽  
pp. 1955-1959 ◽  
Author(s):  
Gurbuz Erdogan ◽  
Recai Pabuccu ◽  
Sibel Ertek ◽  
Shoshana Israel ◽  
Banu Yilmaz ◽  
...  
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Cushing Syndrome ◽  
Late Onset ◽  
Adrenal Hyperplasia

scholarly journals Iatrogenic Cushing Syndrome in a Child With Congenital Adrenal Hyperplasia: Erroneous Compounding of Hydrocortisone

2017 ◽  
Vol 103 (1) ◽  
pp. 7-11 ◽  
Author(s):  
Julia E Barillas ◽  
Daniel Eichner ◽  
Ryan Van Wagoner ◽  
Phyllis W Speiser
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Cushing Syndrome ◽  
Adrenal Hyperplasia

scholarly journals Bilateral Adrenalectomy in Congenital Adrenal Hyperplasia: A Systematic Review and Meta-Analysis

2018 ◽  
Vol 103 (5) ◽  
pp. 1767-1778 ◽  
Author(s):  
Diana MacKay ◽  
Anna Nordenström ◽  
Henrik Falhammar
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Cushing Syndrome ◽  
Case Reports ◽  
Therapeutic Option ◽  
Meta Analysis ◽  
Case Series ◽  
Adverse Outcomes ◽  
Bilateral Adrenalectomy ◽  
Adrenal Hyperplasia

Abstract Context Management of congenital adrenal hyperplasia (CAH) involves suppression of the hypothalamic–pituitary–adrenal axis using supraphysiological doses of exogenous glucocorticoids. This can pose a challenge, with Cushing syndrome a frequent complication of adequate suppression. Bilateral adrenalectomy, with subsequent replacement of glucocorticoids and mineralocorticoids at physiological doses, has been proposed as an alternative therapeutic strategy. Objective To review the outcomes after bilateral adrenalectomy for CAH. Data Sources A systematic search of PubMed/MEDLINE and Web of Science, identifying relevant reports published up to 10 January 2018. Study Selection Case reports or case series were included if they reported individual patient data from patients with CAH who had undergone bilateral adrenalectomy. Data Extraction Information regarding the following was extracted: first author, country, sex, age at adrenalectomy, year of adrenalectomy, diagnosis, molecular abnormality, pre- and postoperative biochemistry, pre- and postoperative medications, pre- and postoperative body mass index, indication for adrenalectomy, surgical technique, gross and microscopic adrenal characteristics, follow-up duration, and short- and long-term postoperative outcomes. Data Synthesis We identified 48 cases of bilateral adrenalectomy for CAH, with patients aged from 4 months to 56 years at surgery. The most common indication for surgery was the inability to control hyperandrogenism/virilization and/or Cushing syndrome (n = 30; 62%). Most patients (n = 34; 71%) reported symptomatic improvement postoperatively, with some cases of short-term (n = 5; 10%) and long-term (n = 13; 27%) adverse outcomes. Conclusions Bilateral adrenalectomy for CAH appears to be a reasonable therapeutic option for carefully selected patients who have had unsatisfactory outcomes with conventional medical management.

scholarly journals POLYCYSTIC OVARIAN DISEASE

2016 ◽  
Vol 23 (07) ◽  
pp. 775-779
Author(s):  
Razia Tarique Qureshi ◽  
Fouzia Rahim ◽  
Gulfreen Haidar
Keyword(s):  
Menstrual Cycle ◽  
Congenital Adrenal Hyperplasia ◽  
Study Design ◽  
Cushing Syndrome ◽  
Clomiphene Citrate ◽  
Menstrual Irregularity ◽  
Adrenal Hyperplasia ◽  
Polycystic Ovarian Disease ◽  
Detailed History

Objectives: To determine the role of metformin in correcting the irregularity ofmenstrual periods and infertility due to PCO. Study Design: Descriptive observational study.Setting: Gynecology Department of PUMHS Nawabshah. Period: 4th February 2010 up toNovember 2011. Patients and methods: Total 329 patients had selected in this series, based onpresence of PCO on ultrasound with 2 or extra following criteria like oligomenorrhea, hirsutism,hyperandrogenism and reversed FSH: LH ratio. Other endocrinal reasons of female infertilityas Cushing syndrome, congenital adrenal hyperplasia, hyperprolectinemia and cases thyroiddisorder had excluded. After detailed history, Metformin was started with dose of 250mgonce a day initially then gradually adjusted to 500mg TD. Weight decreased was encouragedwith diet and the exercise. Cases assessed after six months for checking regularity of theirmenstrual cycle, fertility and the BMI change. Cases those not be success to conceive following6 months, than the ongoing criteria of clomiphene. Women those failed to conceive afteruses of clomiphene citrate, than metformin alone was sustained and fertility had re evaluatedon end of 1 year. Results: 329 cases had selected. Commonest menstrual irregularity wasoligomenorrhea with hypo menorrhea which is seen in 46% of patients. After six months ofmetformin treatment 82% cases assessed improvement of menstrual cycle, while 13% still haveoligomenorrhea. Results found significant (P<0.001) .BMI of all study patients was comparedprior and then treatment and significant results were found (P <0.001). After six months forfertility analysis, complete data was available from 117 patients only. Overall fertility rate was91.1%. Conclusion: Metformin is successful treatment of cases having PCOS.

scholarly journals Congenital Adrenocortical Tumor in an Asymptomatic Neonate with Positive Newborn Screen for Congenital Adrenal Hyperplasia: A Case Report

2017 ◽  
Vol 3 ◽  
pp. 1-4
Author(s):  
Phuong Thao Hoang ◽  
Tsay Eric ◽  
John Mace ◽  
Eba Hathout
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Newborn Screening ◽  
Germ Line ◽  
Cushing Syndrome ◽  
Abdominal Mass ◽  
Laboratory Data ◽  
Abdominal Ultrasound ◽  
Adrenal Hyperplasia ◽  
Li Fraumeni Syndrome ◽  
Palpable Abdominal Mass

Adrenocortical tumors (ACTs) are rare in childhood, particularly in the neonatal period. ACTs in children usually secrete hormones that can cause virilization, precocious puberty, or Cushing syndrome. However, in many cases the diagnosis is delayed because of the generally healthy appearance of the child and the lack of a palpable abdominal mass. ACTs can also be misdiagnosed as congenital adrenal hyperplasia (CAH) especially if 17-hydroxyprogesterone (17-OHP) is elevated. We describe a case of neonatal ACT diagnosed in an asymptomatic newborn with positive/elevated 17-OHP on newborn screening. On examination there were no signs of virilization, Cushing syndrome, hemihypertrophy, or hypertension, and there was no palpable abdominal mass. While awaiting confirmatory results of an elevated 17-OHP level, an abnormally low adrenocorticotropic hormone level was noted. Abdominal ultrasound, obtained to evaluate the adrenal glands, showed a right adrenal mass with peripheral calcification. MRI revealed a 3.6 x 3.5 x 3.4 cm heterogeneously enhancing ovoid mass in the right adrenal gland. Diagnosis of an ACT was also confirmed by laboratory data, and histopathology of the tumor. Surgical resection was safely performed with perioperative steroid replacement. Genetic analysis of CYP21A2 and CYP11B1 genes was negative. A germ-line point mutation in the TP53 region was discovered, suggesting Li-Fraumeni syndrome. ACTs should be considered in the differential diagnosis of neonates with elevated 17-OHP detected by newborn screening for CAH. Adrenal ultrasound is recommended in cases of significantly abnormal CAH screen results to rule out or treat adrenal neoplasms.

926: Dorsal Nerve Preservation in Modified Reduction Clitoroplasty in Congenital Adrenal Hyperplasia (CAH)

2007 ◽  
Vol 177 (4S) ◽  
pp. 307-307
Author(s):  
Ariella Hochsztein ◽  
Rebecca Baergen ◽  
Emily Loyd ◽  
Jie Chen ◽  
Diane Felsen ◽  
...  
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Adrenal Hyperplasia ◽  
Nerve Preservation ◽  
Dorsal Nerve

Abstract #106: Congenital Adrenal Hyperplasia Presenting with Giant Adrenal Myelolipomas

2017 ◽  
Vol 23 ◽  
pp. 4-5
Author(s):  
Tarandeep Kaur ◽  
Peter Dipasco ◽  
Leland Graves
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Adrenal Hyperplasia
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