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Sample records for nonclassic adrenal hyperplasia

  1. Nonclassical Congenital Adrenal Hyperplasia and Pregnancy

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    Neslihan Cuhaci

    2015-01-01

    Full Text Available Objective. The most common form of congenital adrenal hyperplasia (CAH is 21-hydroxylase (21-OH deficiency due to mutation of the CYP21A2 gene. Patients with nonclassical CAH (NC-CAH are usually asymptomatic at birth and typically present in late childhood, adolescence, or adulthood with symptoms of excessive androgen secretion. Subfertility is relative in NC-CAH, but the incidence of spontaneous miscarriage is higher. Here, we report a previously undiagnosed female who gave birth to a normal male child and is planning to become pregnant again. Case Report. A 32-year-old female was referred to our clinic for obesity. Her medical history revealed that she had had three pregnancies. She was planning to become pregnant again. Her laboratory results revealed that she had NC-CAH. Since her husband is the son of her aunt and she had miscarriages and intrauterin exitus in her history, their genetic analyses were performed. Conclusion. Since most patients with NC-CAH have a severe mutation, these patients may give birth to a child with the classical CAH (C-CAH if their partner is also carrying a severe mutation. Females with NC-CAH who desire pregnancy must be aware of the risk of having an infant with C-CAH.

  2. Nonclassic congenital adrenal hyperplasia misdiagnosed as Turner syndrome

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    Vineet V Mishra

    2015-01-01

    Full Text Available We present a patient with nonclassic congenital adrenal hyperplasia (NCAH misdiagnosed as mosaic Turner syndrome. She presented with complaints of primary infertility. Short stature, the presence of facial hair and hoarse voice was also noted. She had primary amenorrhea and was advised for karyotype at 16 years of age, which was reported as 45, X[20]/46, XX[80], stating her as a case of mosaic Turner syndrome. Clitoroplasty was done at 21 years of age for clitoromegaly, which was noticed during puberty. The diagnosis of mosaic Turner could not explain the virilization. Therefore, we repeated the karyotype, which revealed 46, XX in more than 100 metaphases and was sufficient to exclude mosaicism. Furthermore, the endocrinological evaluation revealed high testosterone level with a normal 17 alpha-hydroxyprogesterone (17-OHP. The presence of pubertal onset virilization with a karyotype of 46, XX and raised testosterone level with normal 17-OHP level, raised the suspicion of NCAH for which adrenocorticotropic hormone stimulation test was done which confirmed the diagnosis of NCAH.

  3. [Diffuse hypertrichosis revealing non-classical congenital adrenal hyperplasia].

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    Berthin, C; Sibilia, P; Martins-Hericher, J; Donzeau, A; Martin, L

    2018-03-07

    Non-classical congenital adrenal hyperplasia (NC-CAH) is a recessive autosomal disease caused by a deficiency of adrenal steroidogenesis enzymes. It must be distinguished from classical CAH, either simple virilising or salt-wasting, diagnosed during the neonatal period and responsible for potentially lethal disorders of sexual differentiation. NC-CAH presents a simpler and less specific clinical picture. Herein, we present two cases comprising twin girls consulting for diffuse hypertrichosis. Two 5-year-old twin girls were seen at our consultation for increased pilosity on all four limbs, but with no facial pilosity or synophrys, as well as comedones on the chin. Their height and weight and psychomotor development was normal, with no signs of precocious puberty and no clitoral hypertrophy. Levels of 17OH-P and SDHA were high, while FSH and LH were low and IGF1 and TSH were normal. Analysis of gene CYP21 associated with NC-CAH showed mutations p.V281L and IVS2-13A/C>G. Mutation p.V281L was present in the heterozygous state in the older sister and the father, together with moderate hyperpilosity but without hirsutism or acne. No mutations were found in the mother, indicating either de novo appearance of mutation IVS2-13A/C>G in the twins or germline mosaicism in the mother. We diagnosed NC-CAH as the cause of diffuse hypertrichosis in these twins. This disease is not rare, with a prevalence of 1/1000 to 1500 among peoples of European descent. It is often diagnosed late since routine neonatal screening is not performed. In some cases, NC-CAH remains asymptomatic. The appearance of pubic hair at around 5 to 7 years is the initial reason for consultation, particularly with a dermatologist. Hyperandrogenism varies, involving hirsutism, acne, fertility disorders and premature ageing of bone. Cortisol and aldosterone levels are generally normal. The risk of acute adrenal insufficiency is extremely low. Differential diagnosis concerns ovarian or adrenal tumors and

  4. Ultralow-dose dexamethasone to preserve endogenous cortisol stress response in nonclassical congenital adrenal hyperplasia: A new promising treatment

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    D.C.M. van der Kaay (Danielle); E.L.T. van den Akker (Erica)

    2014-01-01

    textabstractIntroduction: Nonclassical congenital adrenal hyperplasia (CAH) is characterized by sufficient cortisol and aldosterone production at the cost of androgen overproduction. Hydrocortisone or dexamethasone in supraphysiological doses are current treatment; however, their downside is

  5. Revisiting the prevalence of nonclassic congenital adrenal hyperplasia in US Ashkenazi Jews and Caucasians.

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    Hannah-Shmouni, Fady; Morissette, Rachel; Sinaii, Ninet; Elman, Meredith; Prezant, Toni R; Chen, Wuyan; Pulver, Ann; Merke, Deborah P

    2017-11-01

    PurposeNonclassic 21-hydroxylase deficiency, a mild form of congenital adrenal hyperplasia (CAH), is estimated to be the most common autosomal recessive condition, with an especially high prevalence in Ashkenazi Jews (3.7% affected, 30.9% carriers), based on a 1985 HLA-B linkage study of affected families. Affected individuals, especially women, may suffer from hyperandrogenism and infertility. State-of-the-art genetic studies have not been done to confirm these remarkable rates.MethodsCYP21A2 genotyping was performed in 200 unrelated healthy Ashkenazi Jewish subjects and 200 random US Caucasians who did not self-identify as a specific ethnicity using multiplex minisequencing, real-time polymerase chain reaction and junction site analysis.ResultsNonclassic CAH carriership was found similarly in 15% (95% confidence interval (CI): 10.4-20.7) of Ashkenazi Jews and 9.5% (95% CI: 5.8-14.4) of Caucasians (P=0.13). The proportion of Ashkenazi Jewish nonclassic CAH carriers (0.15 versus 0.309, Pcounseling.

  6. Characterization of novel StAR (steroidogenic acute regulatory protein mutations causing non-classic lipoid adrenal hyperplasia.

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    Christa E Flück

    Full Text Available CONTEXT: Steroidogenic acute regulatory protein (StAR is crucial for transport of cholesterol to mitochondria where biosynthesis of steroids is initiated. Loss of StAR function causes lipoid congenital adrenal hyperplasia (LCAH. OBJECTIVE: StAR gene mutations causing partial loss of function manifest atypical and may be mistaken as familial glucocorticoid deficiency. Only a few mutations have been reported. DESIGN: To report clinical, biochemical, genetic, protein structure and functional data on two novel StAR mutations, and to compare them with published literature. SETTING: Collaboration between the University Children's Hospital Bern, Switzerland, and the CIBERER, Hospital Vall d'Hebron, Autonomous University, Barcelona, Spain. PATIENTS: Two subjects of a non-consanguineous Caucasian family were studied. The 46,XX phenotypic normal female was diagnosed with adrenal insufficiency at the age of 10 months, had normal pubertal development and still has no signs of hypergonodatropic hypogonadism at 32 years of age. Her 46,XY brother was born with normal male external genitalia and was diagnosed with adrenal insufficiency at 14 months. Puberty was normal and no signs of hypergonadotropic hypogonadism are present at 29 years of age. RESULTS: StAR gene analysis revealed two novel compound heterozygote mutations T44HfsX3 and G221S. T44HfsX3 is a loss-of-function StAR mutation. G221S retains partial activity (∼30% and is therefore responsible for a milder, non-classic phenotype. G221S is located in the cholesterol binding pocket and seems to alter binding/release of cholesterol. CONCLUSIONS: StAR mutations located in the cholesterol binding pocket (V187M, R188C, R192C, G221D/S seem to cause non-classic lipoid CAH. Accuracy of genotype-phenotype prediction by in vitro testing may vary with the assays employed.

  7. Increased large artery intima media thickness in adolescents with either classical or non-classical congenital adrenal hyperplasia.

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    Wasniewska, M; Balsamo, A; Valenzise, M; Manganaro, A; Faggioli, G; Bombaci, S; Conti, V; Ferri, M; Aversa, T; Cicognani, A; De Luca, F

    2013-01-01

    Increased artery intima-media thickness (IMT) was found in adults with classical congenital adrenal hyperplasia (CAH). No data are available in patients with non-classical (NC) CAH. To evaluate IMT in adolescents with classical and NC CAH and to compare the results with those recorded in a control population. Eighteen adolescents with either classical (Subgroup A1) or NC CAH (Subgroup A2) were compared with 16 controls (Group B). All subjects underwent IMT ultrasonography measurement at different sites; results were correlated with clinical, metabolic, and insulin resistance (IR) data. When compared with Group B, both subgroups exhibited higher IMT values at all sites. No differences were found between classical and NC CAH. Univariate analysis of factors impacting on IMT of CAH patients demonstrated that: a) abdominal aorta (AA) IMT was positively correlated with cumulative glucocorticoid doses, triglyceride serum levels, and diastolic blood pressure SD score and negatively with androstenendione and ACTH levels; b) common carotid (CC) IMT was positively associated with triglycerides and triglyceride/HDL ratio. At multiple regression analysis, the independent positive predictors of AA and CC IMT were respectively triglyceride levels and triglyceride/HDL ratio. a) Even adolescents with NC CAH and not only those with classical form may be at higher risk of artery alterations; b) this risk is not necessarily associated with either obesity or waist/height ratio or dyslipidemia; c) an important role in the pathogenesis of artery alterations in CAH may be played by intermittent iatrogenic hypercortisolism and secondary IR.

  8. Congenital adrenal hyperplasia: Case report.

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    Jaime Avaria E.; María José Vargas F.; Loreto Triviño F.; Andrea Gleisner E.

    2013-01-01

    INTRODUCTION: Congenital adrenal hyperplasia (CAH) is an autosomal recessive disease whose main cause is the deficiency of 21-hydroxylase, an enzyme involved in the synthesis of cortisol and aldosterone. There are two forms of CAH, a classical and nonclassical form, being the first objective of analysis in the clinical case. Its clinical manifestations vary in severity, depending on the level of hormone deficiency. Within the classic is described the salt-wasting form, whose consequences are ...

  9. [Successful treatment and pregnancy in a women with the non-classic form of congenital adrenal hyperplasia

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    Akker, E. van den; Stikkelbroeck, M.M.L.; Menheere, P.P.C.A.; Roumen, F.J.M.E.; Otten, B.J.

    2002-01-01

    In an 18-year-old woman non-classic 21-hydroxylase deficiency was diagnosed and dexamethasone treatment was instituted. Ten years later, she became pregnant for the first time; at 37 weeks unexpected intrauterine foetal death was found to have occurred. A second pregnancy ended with a spontaneous

  10. Genetics Home Reference: primary macronodular adrenal hyperplasia

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    ... Support and Research Foundation: Genetic Changes Found in Cushing's Disease, Adrenal Tumors, and Adrenal Hyperplasia MalaCards: acth-independent ... macronodular adrenal hyperplasia 2 Merck Manual (Home Edition): Cushing ... Adrenal Diseases Foundation: Cushing's Syndrome Orphanet: Cushing syndrome due to ...

  11. Congenital Adrenal Hyperplasia

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    ... Español NICHD Theme Browse AZTopics Browse A-Z Adrenal Gland Disorders Autism Spectrum Disorder (ASD) Down Syndrome Endometriosis Learning ... Funding Opportunities & Notices Health A to Z List Adrenal Gland Disorders About NICHD Research Information Find a Study More ...

  12. Forma no clásica de hiperplasia adrenal congénita en la niñez y adolescencia Non-classic way of congenital adrenal hyperplasia in childhood and adolescence

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    Francisco Carvajal Martínez

    2010-04-01

    Full Text Available INTRODUCCIÓN: la hiperplasia adrenal congénita es un trastorno hereditario de la esteroidogénesis suprarrenal, trasmitido por mutaciones genéticas con carácter autosómico recesivo, las cuales afectan las enzimas que intervienen en la biosíntesis del cortisol. La causa la constituye en 90 a 95 % de los casos la deficiencia de la enzima 21 hidroxilasa. OBJETIVO: exponer la experiencia de los autores de este trabajo en la forma no clásica de esta enfermedad. MÉTODOS: se realizó la caracterización de 7 pacientes diagnosticados en la sala de endocrinología pediátrica del Instituto Nacional de Endocrinología, ubicada en el Hospital Pediátrico del Cerro, durante el período 1998-2008. Todos los pacientes pertenecían al sexo femenino. RESULTADOS: los síntomas se iniciaron a una edad promedio de 8,8 años y más de la mitad de los casos presentaron pubarquia precoz. La menarquia se produjo a una edad media de 10,7 años. Se logró el diagnóstico bioquímico al obtenerse valores elevados de 17 hidroxiprogesterona (en condiciones basales. Se emplearon distintas modalidades de tratamiento según la edad de cada paciente y los síntomas predominantes en cada caso. CONCLUSIONES: se corroboró la mayor frecuencia de diagnóstico de esta enfermedad en el sexo femenino, así como la importancia del estudio y el seguimiento ante un paciente con pubarquia precoz.INTRODUCTION: the congenital adrenal hyperplasia is an inherited disorder of suprarenal esteroidogenesis, transmitted by genetic mutations with a autosomal recessive character affecting the enzymes intervening in cortisol biosynthesis. In the 90 to 95% of cases, the cause is a deficiency of Hydroxylase enzyme 21. OBJECTIVE: to show the current paper authors' experience in the non-classic way of this entity. METHODS: we made a characterization of 7 female patients diagnosed in the Children Endocrinology Ward of the National Institute of Endocrinology located in the Children Hospital, Cerro

  13. Unilateral nodular adrenal hyperplasia: Case series

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    A.F. Kotb

    2016-07-26

    Jul 26, 2016 ... Abstract. Introduction: Nodular adrenal hyperplasia is one of rare causes of adrenocortical hyperplasia. The disease usually presents bilaterally. Few publications discussed the possibility of unilateral disease, in association with hyperaldosteronism or Cushing syndrome. Case series: We are reporting 3 ...

  14. Giant Bilateral Adrenal Myelolipoma with Congenital Adrenal Hyperplasia

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    S. Al-Bahri

    2014-01-01

    Full Text Available Myelolipomas are rare and benign neoplasms, predominant of the adrenal glands, consisting of adipose and mature hematopoietic tissue, commonly discovered incidentally with increased use of radiologic imaging. Few cases of giant bilateral adrenal masses are reported, especially in the setting of congenital adrenal hyperplasia (CAH. We report the case of a 39-year-old male with a history of CAH secondary to 21-α hydroxylase deficiency on steroids since childhood, self-discontinued during adolescence, presenting with abdominal distension, fatigue, decreased libido, and easy bruising. Imaging revealed giant bilateral adrenal masses. He subsequently underwent bilateral adrenalectomy found to be myelolipomas measuring 30 × 25 × 20 cm on the left and weighing 4.1 kg and 25 × 20 × 13 cm on the right and weighing 2.7 kg. Adrenal myelolipomas are found to coexist with many other conditions such as Cushing’s syndrome, Addison’s disease, and CAH. We discuss the association with high adrenocorticotropic hormone (ACTH states and review the studies involving ACTH as proponent leading to myelolipomas. Massive growth of these tumors, as in our case, can produce compression and hemorrhagic symptoms. We believe it is possible that self-discontinuation of steroids, in the setting of CAH, may have resulted in the growth of his adrenal masses.

  15. Adrenal Hypoplasia Congenita Presenting as Congenital Adrenal Hyperplasia

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    Jennifer L. Flint

    2013-01-01

    Full Text Available We report on a patient with genetically confirmed adrenal hypoplasia congenita (AHC whose presentation and laboratory abnormalities were consistent with the more common condition, congenital adrenal hyperplasia (CAH. The patient presented with failure to thrive and salt wasting. General appearance showed marked hyperpigmentation and normal male genitalia. He displayed mildly elevated 17-hydroxyprogesterone and markedly elevated 11-deoxycortisol levels at baseline and with ACTH stimulation testing. Results were consistent with 11β-hydroxylase deficiency. He required glucocorticoids and high doses of mineralocorticoids. The marked elevation in 11-deoxycortisol directed our clinical reasoning away from a hypoplastic condition and towards a hyperplasic adrenal condition. Sequencing of the DAX1 gene (named for dosage-sensitive sex reversal (DSS locus and the AHC locus on the X chromosome revealed a missense mutation. A review of the literature revealed that elevated 11-deoxycortisol levels have been noted in kindreds with DAX1 mutations, but only when measured very early in life. A mouse model has recently been described that displays elevated 11-deoxycorticosterone levels and evidence for hyperplasia of the zona glomerulosa of the adrenal gland. We conclude that DAX1 testing may be considered in patients with laboratory evidence of 11β-hydroxylase deficiency, especially in those with severe salt wasting.

  16. Impact of molecular genetics on congenital adrenal hyperplasia management.

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    Balsamo, A; Baldazzi, L; Menabò, S; Cicognani, A

    2010-09-01

    Congenital adrenal hyperplasia (CAH) is a family of autosomal recessive disorders caused by mutations in genes encoding the enzymes involved in one of the 5 steps of adrenal steroid synthesis or the electron donor P450 oxidoreductase (POR) enzyme. Steroid 21-hydroxylase deficiency (21-OHD), the principal focus of this review, accounts for about 90-95% of all CAH cases, and its biochemical and clinical severity depends on the underlying CYP21A2 gene disruption. Molecular genetic advancements have been achieved in recent years, and the aim of this review is to attempt to highlight its contribution to the comprehension and management of the disease. When possible, we will try to achieve this goal also by providing some results from our personal experience regarding: some aspects of CYP21A2 gene analysis, with basic genotype/phenotype relationships; its crucial role in both genetic counselling and in prenatal diagnosis and treatment in families at risk for 21-OHD; its help in the comprehension of the severity of the disease in patients diagnosed by neonatal screening and possibly treated before an evident salt-loss crisis or before performing adequate blood sampling; its usefulness in the definition of post ACTH 17-hydroxyprogesterone values, discriminating between non-classic, heterozygote and normal subjects; and finally the contribution of genes other than CYP21A2 whose function or dysfunction could influence 21-hydroxylase activity and modify the presentation or management of the disease.

  17. Radiological findings of congenital lipoid adrenal hyperplasia: a case report

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    Kim, Mi Jeong; Shin, Joo Yong; Lee, Hee Jung; Lee, Jin Hee; Sohn, Cheol Ho; Lee, Sung Moon; Kim, Hong; Woo, Seong Ku; Suh, Soo Ji

    2001-01-01

    Congenital lipoid adrenal hyperplasia (CLAH) is a rare autosomal recessive disorder characterized by the marked accumulation of lipids and cholesterol in the adrenal cortex, and the failure of adrenal steroids to synthesise. We report the ultrasound (US), computed tomographic (CT), and magnetic resonance (MR) imaging findings in a four-day-old female neonate with CLAH

  18. Congenital adrenal hyperplasia: Treatment and outcomes

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    Mahdi Kamoun

    2013-01-01

    Full Text Available Congenital adrenal hyperplasia (CAH describes a group of autosomal recessive disorders where there is impairment of cortisol biosynthesis. CAH due to 21-hydroxylase deficiency accounts for 95% of cases and shows a wide range of clinical severity. Glucocorticoid and mineralocorticoid replacement therapies are the mainstays of treatment of CAH. The optimal treatment for adults with CAH continues to be a challenge. Important long-term health issues for adults with CAH affect both men and women. These issues may either be due to the disease or to steroid treatment and may affect final height, fertility, cardiometabolic risk, bone metabolism, neuro-cognitive development and the quality-of-life. Patients with CAH should be regularly followed-up from childhood to adulthood by multidisciplinary teams who have knowledge of CAH. Optimal replacement therapy, close clinical and laboratory monitoring, early life-style interventions, early and regular fertility assessment and continuous psychological management are needed to improve outcome.

  19. Unilateral nodular adrenal hyperplasia: Case series | Kot | African ...

    African Journals Online (AJOL)

    Introduction: Nodular adrenal hyperplasia is one of rare causes of adrenocortical hyperplasia. The disease usually presents bilaterally. Few publications discussed the possibility of unilateral disease, in association with hyperaldosteronism or Cushing syndrome. Case series: We are reporting 3 cases of radiologically ...

  20. Congenital adrenal hyperplasia - experience from a tertiary centre in South India

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    George Belinda

    2012-01-01

    Full Text Available Congenital adrenal hyperplasia is a group of autosomal recessive disorders caused by enzyme deficiency which leads to defects in biosynthesis of steroid precursors. Most common is 21 hydroxylase deficiency. Clinical spectrum varies from non-classical CAH to classic CAH, and it may be simple virilising form or salt-wastinfg type. 29 patients were included in our study from January 2012 to October 2012. 76% were females. Male babies typically presented with adrenal crisis between 3 rd to 6 th week of life. Around 20% of females were identified and appropriately treated only after late adolescence. Short stature was seen in 1/3 rd of patients. 1/3 rd of patients had suppressed 17 OHP levels suggestive of over-replacement therapy which may contribute to final reduction in adult height.

  1. Testicular Adrenal Rest Tumors (TARTS With Unusual Histological Features in Congenital Adrenal Hyperplasia (CAH

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    Valeri Marianovsky

    2015-07-01

    Full Text Available Congenital adrenal hyperplasia (CAH patients with testicular adrenal rest tumors (TARTs with testicular enlargement present a serious diagnostic challenge. According to the data TARTs are usually benign. They are rare, resulting in paucity in the medical literature regarding their pathological features. We report a case of bilateral synchronous mass-forming TARTs with marked cytological and nuclear atypia misinterpreted as malignant testicular tumors in a 40-years-old man with CAH and CT and MRI data for pheochromocytoma of the right adrenal gland and paraaortal and paracaval lymphadenomegaly. He was previously diagnosed with adrenal cortical carcinoma of the left adrenal gland.

  2. The role of imaging in congenital adrenal hyperplasia

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    Teixeira, Sara Reis; Andrade, Marco Tulio Soares; Melo, Andrea Farias; Elias Junior, Jorge; Elias, Paula Condé Lamparelli

    2014-01-01

    Congenital adrenal hyperplasia (CAH) is an autossomic recessive disorder caused by impaired steroidogenesis. Patients with CAH may present adrenal insufficiency with or without salt-wasting, as well as various degrees of virilization and fertility impairment, carrying a high incidence of testicular adrenal rest tumors and increased incidence of adrenal tumors. The diagnosis of CAH is made based on the adrenocortical profile hormonal evaluation and genotyping, in selected cases. Follow-up is mainly based on hormonal and clinical evaluation. Utility of imaging in this clinical setting may be helpful for the diagnosis, management, and follow-up of the patients, although recommendations according to most guidelines are weak when present. Thus, the authors aimed to conduct a narrative synthesis of how imaging can help in the management of patients with CAH, especially focused on genitography, ultrasonography, computed tomography, and magnetic resonance imaging. (author)

  3. The role of imaging in congenital adrenal hyperplasia

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    Teixeira, Sara Reis; Andrade, Marco Tulio Soares; Melo, Andrea Farias; Elias Junior, Jorge, E-mail: jejunior@fmrp.usp.br [Department of Internal Medicine, Division of Radiology, Clinical Hospital, Ribeirao Preto Medical School, University of Sao Paulo (FMRP-USP), Ribeirao Preto, SP (Brazil); Elias, Paula Condé Lamparelli [Department of Internal Medicine, Division of Endocrinology, Clinical Hospital, FMRP-USP, Ribeirao Preto, SP (Brazil)

    2014-10-15

    Congenital adrenal hyperplasia (CAH) is an autossomic recessive disorder caused by impaired steroidogenesis. Patients with CAH may present adrenal insufficiency with or without salt-wasting, as well as various degrees of virilization and fertility impairment, carrying a high incidence of testicular adrenal rest tumors and increased incidence of adrenal tumors. The diagnosis of CAH is made based on the adrenocortical profile hormonal evaluation and genotyping, in selected cases. Follow-up is mainly based on hormonal and clinical evaluation. Utility of imaging in this clinical setting may be helpful for the diagnosis, management, and follow-up of the patients, although recommendations according to most guidelines are weak when present. Thus, the authors aimed to conduct a narrative synthesis of how imaging can help in the management of patients with CAH, especially focused on genitography, ultrasonography, computed tomography, and magnetic resonance imaging. (author)

  4. Clinical, Chromosomal and Endocrine Studies for Congenital Adrenal Hyperplasia

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    Shousha, M.A.; Somaya, E.T.; Attia, M.

    2007-01-01

    Several forms of congenital adrenal hyperplasia are potentially fatal if unrecognized and untreated. The aim of this study is to throw light on the clinical presentation together with chromosomal and laboratory associations in this syndrome. Twenty four patients diagnosed as congenital adrenal hyperplasia were referred from the Diabetic Endocrine Metabolic Pediatric Unit [DEMPU], Children's Hospital, Cairo University for hormonal and chromosomal workup. Twenty two patients were diagnosed as classic congenital adrenal hyperplasia (CAH) syndrome. Sever salt wasting form was present in ten patients whereas simple virilization was the presenting manifestation in twelve patients. Two patients presented as late onset congenital adrenal hyperplasia (LOCAH). The mean age was 3.18 years, ranging from eight months to 19 years. The sex of rearing was Female in 18 cases and male in six cases. Genitography and sonography confirmed the presence of female internal organs in all cases. Advanced bone age was evident by radiographic studies. Although the karyotyping was (46,XX) in all cases, the correct sex diagnosis was delayed in 6 cases. Serum concentrations of 17-hydroxyprogesterone (17.OH.P); Dehydroepiandrosterone sulfate (DHEAS); Delta,4-androstenedione (D 4 A); Testosterone and 11-deoxycortisol were all elevated in relation to controls. We found that the adrenal androgens DHEAS, delta 4A, and T were more elevated in salt losers when compared to simple virilizing patients. However, this difference was not of statistical significance. The present study demonstrates that clinical examination and laboratory investigations are necessary for early detection and treatment of hese cases to avoid major medical and psychological problems for the patients and their parents.

  5. ADRENAL CRISIS IN FEMALE NEWBORN WITH UNRECOGNIZED CONGENITAL ADRENAL HYPERPLASIA – CASE REPORT

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    Starčević, Mirta; La Grasta Sabolić, Lavinia

    2008-01-01

    Summary. Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is the most frequent cause of genital ambiguity in female newborns. Enzyme deficiency causes a disorder of cortisol synthesis by the adrenal cortex and leads to excessive androgen production resulting in subsequent genital virilization of female fetuses. The incidence of the classical form is 1:10000–15000 live births. About ¾ of affected infants also suffer from a disorder of aldosterone biosynthesis, which can le...

  6. Diagnosis of adrenal adenoma and hyperplasia by CT and adrenal scintigraphy

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    Miura, Kentaro; Itami, Jun; Nawano, Shigeru; Okada, Junichi; Ogino, Takashi; Uno, Koichi; Arimizu, Noboru

    1985-01-01

    The evaluation of X-CT and adrenal scintigraphy in diagnosis of Cushing syndrome and primary aldosteronism was studied in 18 patients. In Cushing syndrome, CT appearance of adenoma is commonly larger than that of primary aldosteronism and cleary deliniated by surrounding fat. So, in Cushing syndrome, diagnosis of adenoma on CT is much easier than that of primary aldosteronism, and absence of adenoma on CT suggests adrenal hyperplasia. In primary aldosteronism both of CT and scintigraphy must be performed. (author)

  7. Physiological basis for the etiology, diagnosis, and treatment of adrenal disorders: Cushing's syndrome, adrenal insufficiency, and congenital adrenal hyperplasia.

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    Raff, Hershel; Sharma, Susmeeta T; Nieman, Lynnette K

    2014-04-01

    The hypothalamic-pituitary-adrenal (HPA) axis is a classic neuroendocrine system. One of the best ways to understand the HPA axis is to appreciate its dynamics in the variety of diseases and syndromes that affect it. Excess glucocorticoid activity can be due to endogenous cortisol overproduction (spontaneous Cushing's syndrome) or exogenous glucocorticoid therapy (iatrogenic Cushing's syndrome). Endogenous Cushing's syndrome can be subdivided into ACTH-dependent and ACTH-independent, the latter of which is usually due to autonomous adrenal overproduction. The former can be due to a pituitary corticotroph tumor (usually benign) or ectopic ACTH production from tumors outside the pituitary; both of these tumor types overexpress the proopiomelanocortin gene. The converse of Cushing's syndrome is the lack of normal cortisol secretion and is usually due to adrenal destruction (primary adrenal insufficiency) or hypopituitarism (secondary adrenal insufficiency). Secondary adrenal insufficiency can also result from a rapid discontinuation of long-term, pharmacological glucocorticoid therapy because of HPA axis suppression and adrenal atrophy. Finally, mutations in the steroidogenic enzymes of the adrenal cortex can lead to congenital adrenal hyperplasia and an increase in precursor steroids, particularly androgens. When present in utero, this can lead to masculinization of a female fetus. An understanding of the dynamics of the HPA axis is necessary to master the diagnosis and differential diagnosis of pituitary-adrenal diseases. Furthermore, understanding the pathophysiology of the HPA axis gives great insight into its normal control. © 2014 American Physiological Society.

  8. Diagnosis and management of classical congenital adrenal hyperplasia.

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    Marumudi, Eunice; Khadgawat, Rajesh; Surana, Vineet; Shabir, Iram; Joseph, Angela; Ammini, Ariachery C

    2013-08-01

    Congenital adrenal hyperplasia (CAH) is among the most common genetic disorders. Deficiency of adrenal steroid 21-hydroxylase deficiency due to mutations in the CYP21A2 gene accounts for about 95% cases of CAH. This disorder manifests with androgen excess with or without salt wasting. It also is a potentially life threatening disorder; neonatal screening with 17-hydroxyprogesterone measurement can diagnose the condition in asymptomatic children. Carefully monitored therapy with glucocorticoid and mineralocorticoid supplementation will ensure optimal growth and development for children with CAH. Genital surgery may be required for girls with CAH. Continued care is required for individuals with CAH as adults to prevent long-term adverse consequences of the disease, including infertility, metabolic syndrome and osteoporosis. Copyright © 2013 Elsevier Inc. All rights reserved.

  9. Growth and Reproductive Outcomes in Congenital Adrenal Hyperplasia

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    Todd D. Nebesio

    2010-01-01

    Full Text Available The treatment of congenital adrenal hyperplasia (CAH due to 21-hydroxylase deficiency is complex. In addition to disease control, important therapeutic goals are the maintenance of normal growth and the acquisition of normal reproductive function. Here, data regarding final adult height (FH in patients with CAH will be reviewed. Additional difficulties associated with CAH, including risks of obesity and hypertension, will be discussed. Information about fertility and reproductive outcomes in men and women with CAH will also be summarized. Although the treatment of each child with CAH needs to be individualized, close medical followup and laboratory monitoring along with good compliance can often result in positive clinical outcomes.

  10. Recent advances in biochemical and molecular analysis of congenital adrenal hyperplasia due to 21-hydroxylase deficiency

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    Jin-Ho Choi

    2016-03-01

    Full Text Available The term congenital adrenal hyperplasia (CAH covers a group of autosomal recessive disorders caused by defects in one of the steroidogenic enzymes involved in the synthesis of cortisol or aldosterone from cholesterol in the adrenal glands. Approximately 95% of all CAH cases are caused by 21-hydroxylase deficiency encoded by the CYP21A2 gene. The disorder is categorized into classical forms, including the salt-wasting and the simple virilizing types, and nonclassical forms based on the severity of the disease. The severity of the clinical features varies according to the level of residual 21-hydroxylase activity. Newborn screening for CAH is performed in many countries to prevent salt-wasting crises in the neonatal period, to prevent male sex assignment in affected females, and to reduce long-term morbidities, such as short stature, gender confusion, and psychosexual disturbances. 17α-hydroxyprogesterone is a marker for 21-hydroxylase deficiency and is measured using a radioimmunoassay, an enzyme-linked immunosorbent assay, or a fluoroimmunoassay. Recently, liquid chromatography linked with tandem mass spectrometry was developed for rapid, highly specific, and sensitive analysis of multiple analytes. Urinary steroid analysis by gas chromatography mass spectrometry also provides qualitative and quantitative data on the excretion of steroid hormone metabolites. Molecular analysis of CYP21A2 is useful for genetic counseling, confirming diagnosis, and predicting prognoses. In conclusion, early detection using neonatal screening tests and treatment can prevent the worst outcomes of 21-hydroxylase deficiency.

  11. Modeling Congenital Adrenal Hyperplasia and Testing Interventions for Adrenal Insufficiency Using Donor-Specific Reprogrammed Cells

    Directory of Open Access Journals (Sweden)

    Gerard Ruiz-Babot

    2018-01-01

    Full Text Available Adrenal insufficiency is managed by hormone replacement therapy, which is far from optimal; the ability to generate functional steroidogenic cells would offer a unique opportunity for a curative approach to restoring the complex feedback regulation of the hypothalamic-pituitary-adrenal axis. Here, we generated human induced steroidogenic cells (hiSCs from fibroblasts, blood-, and urine-derived cells through forced expression of steroidogenic factor-1 and activation of the PKA and LHRH pathways. hiSCs had ultrastructural features resembling steroid-secreting cells, expressed steroidogenic enzymes, and secreted steroid hormones in response to stimuli. hiSCs were viable when transplanted into the mouse kidney capsule and intra-adrenal. Importantly, the hypocortisolism of hiSCs derived from patients with adrenal insufficiency due to congenital adrenal hyperplasia was rescued by expressing the wild-type version of the defective disease-causing enzymes. Our study provides an effective tool with many potential applications for studying adrenal pathobiology in a personalized manner and opens venues for the development of precision therapies.

  12. Ectopic adrenal rests in congenital adrenal hyperplasia as a cause of androgen excess after adrenalectomy detected by pelvic venous sampling.

    NARCIS (Netherlands)

    Claahsen-van der Grinten, H.L.; Stikkelbroeck, M.M.L.; Bulten, J.; Heyer, M. den

    2013-01-01

    BACKGROUND: Patients with classic congenital adrenal hyperplasia (CAH) due to CYP21 deficiency are treated with supraphysiological doses of glucocorticoids to suppress elevated androgen production. This implies also side effects of high-dose glucocorticoids, possibly leading to iatrogenic Cushing's

  13. Mineralocorticoid Deficiency and Treatment in Congenital Adrenal Hyperplasia

    Directory of Open Access Journals (Sweden)

    Padidela Raja

    2010-04-01

    Full Text Available Approximately 75%–80% of patients with Congenital Adrenal Hyperplasia (CAH fail to synthesize sufficient mineralocorticoids to maintain salt and water balance. In most instances genotype can predict mineralocorticoid deficiency in CAH. Early recognition and replacement with 9-fludrocortisone and salt supplements will prevent development of potentially lethal salt losing crises. In infancy a relative state of aldosterone resistance exists and replacement dose of 9-fludrocortisone based on body surface area is higher during infancy compared to childhood and adults. Salt supplementation is generally not required after weaning is started. Regular monitoring of blood pressure and measurements of plasma electrolytes and renin are required to prevent complications of under or over dosage.

  14. The Role of gsp Mutations on the Development of Adrenal Cortical Tumors and Adrenal Hyperplasias

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    Maria Candida Barisson Villares Fragoso

    2016-07-01

    Full Text Available Somatic GNAS point mutations, commonly known as gsp mutations, are involved in the pathogenesis of McCune Albright syndrome and have also been described in autonomous hormone-producing tumors, such as somatotropinoma, corticotrophoma, thyroid cancer, ovarian and testicular Leydig cell tumors and primary macronodular adrenocortical hyperplasia (PMAH. [1-3]The involvement of gsp mutations in adrenal tumors was first described by Lyons et al. in 1990. Since then, several studies have detected the presence of gsp mutations in adrenal tumors, but none of them could explain its presence along or the mechanism that leads to tumor formation and hormone hypersecretion. As a result, the molecular pathogenesis of the majority of sporadic adrenocortical tumors remains unclear. [3] PMAH has also been reported with gsp somatic mutations in a few cases. Fragoso et al. in 2003 identified two distinct gsp somatic mutations affecting arginine residues on codon 201 of GNAS in a few patients with PMAH who lacked any features or manifestations of McCune Albright syndrome. Followed by this discovery, other studies have continued looking for gsp mutations based on strong prior evidence demonstrating that increased cAMP signaling is sufficient for cell proliferation and cortisol production. [2, 4] With consideration for the previously reported findings, we conjecture that although somatic activating mutations in GNAS are a rare molecular event, these mutations could probably be sufficient to induce the development of macronodule hyperplasia and variable cortisol secretion.In this manuscript, we revised the presence of gsp mutations associated with adrenal cortical tumors and hyperplasia.

  15. Decreased adrenomedullary function in infants with classical congenital adrenal hyperplasia.

    Science.gov (United States)

    Kim, Mimi S; Ryabets-Lienhard, Anna; Bali, Bhavna; Lane, Christianne J; Park, Ashley H; Hall, Sandra; Geffner, Mitchell E

    2014-08-01

    Classical congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency can cause life-threatening adrenal crises as well as severe hypoglycemia, especially in very young children. Studies of CAH patients 4 years old or older have found abnormal morphology and function of the adrenal medulla and lower levels of epinephrine and glucose in response to stress than in controls. However, it is unknown whether such adrenomedullary abnormalities develop in utero and/or exist during the clinically high-risk period of infancy and early childhood. The objective of the study was to characterize adrenomedullary function in infants with CAH by comparing their catecholamine levels with controls. Design/Settings: This was a prospective cross-sectional study in a pediatric tertiary care center. Plasma epinephrine and norepinephrine levels were measured by HPLC. Infants with CAH (n = 9, aged 9.6 ± 11.4 d) had significantly lower epinephrine levels than controls [n = 12, aged 7.2 ± 3.2 d: median 84 [(25th; 75th) 51; 87] vs 114.5 (86; 175.8) pg/mL, respectively (P = .02)]. Norepinephrine to epinephrine ratios were also significantly higher in CAH patients than controls (P = .01). The control infants had primary hypothyroidism, but pre- and posttreatment analyses revealed no confounding effects on catecholamine levels. This study demonstrates for the first time that infants with classical CAH due to 21-hydroxylase deficiency have significantly lower plasma epinephrine levels than controls, indicating that impaired adrenomedullary function may occur during fetal development and be present from birth. A longitudinal study of adrenomedullary function in CAH patients from infancy through early childhood is warranted.

  16. Functional analysis of human cytochrome P450 21A2 variants involved in congenital adrenal hyperplasia

    Energy Technology Data Exchange (ETDEWEB)

    Wang, Chunxue; Pallan, Pradeep S.; Zhang, Wei; Lei, Li; Yoshimoto, Francis K.; Waterman, Michael R.; Egli, Martin; Guengerich, F. Peter (Vanderbilt-MED)

    2017-05-24

    Cytochrome P450 (P450, CYP) 21A2 is the major steroid 21-hydroxylase, converting progesterone to 11-deoxycorticosterone and 17α-hydroxyprogesterone (17α-OH-progesterone) to 11-deoxycortisol. More than 100 CYP21A2 variants give rise to congenital adrenal hyperplasia (CAH). We previously reported a structure of WT human P450 21A2 with bound progesterone and now present a structure bound to the other substrate (17α-OH-progesterone). We found that the 17α-OH-progesterone- and progesterone-bound complex structures are highly similar, with only some minor differences in surface loop regions. Twelve P450 21A2 variants associated with either salt-wasting or nonclassical forms of CAH were expressed, purified, and analyzed. The catalytic activities of these 12 variants ranged from 0.00009% to 30% of WT P450 21A2 and the extent of heme incorporation from 10% to 95% of the WT. Substrate dissociation constants (Ks) for four variants were 37–13,000-fold higher than for WT P450 21A2. Cytochrome b5, which augments several P450 activities, inhibited P450 21A2 activity. Similar to the WT enzyme, high noncompetitive intermolecular kinetic deuterium isotope effects (≥ 5.5) were observed for all six P450 21A2 variants examined for 21-hydroxylation of 21-d3-progesterone, indicating that C–H bond breaking is a rate-limiting step over a 104-fold range of catalytic efficiency. Using UV-visible and CD spectroscopy, we found that P450 21A2 thermal stability assessed in bacterial cells and with purified enzymes differed among salt-wasting- and nonclassical-associated variants, but these differences did not correlate with catalytic activity. Our in-depth investigation of CAH-associated P450 21A2 variants reveals critical insight into the effects of disease-causing mutations on this important enzyme.

  17. Complex Genital Malformation in a Female with Congenital Adrenal Hyperplasia: Evaluation with Magnetic Resonance Imaging

    International Nuclear Information System (INIS)

    Klessen, C.; Asbach, P.; Hein, P. A.; Beyersdorff, D.; Hamm, B.; Taupitz, M.

    2005-01-01

    This is a case of complex genital malformation in a young patient with congenital adrenal hyperplasia. The magnetic resonance imaging (MRI) findings included ostium of the vagina into the urethra (common urogenital opening), prostate-like tissue surrounding the urethra, and hyperplasia of the left adrenal gland. The report provides information on the clinical findings, the MRI examination, including the applied sequences and the MR findings, and gives an overview of the disease pattern and its frequency of occurrence

  18. Parental management of adrenal crisis in children with congenital adrenal hyperplasia.

    Science.gov (United States)

    Fleming, Louise; Knafl, Kathleen; Knafl, George; Van Riper, Marcia

    2017-10-01

    Congenital adrenal hyperplasia (CAH) requires parents to inject their child with hydrocortisone intramuscularly during times of illness and adrenal crisis. The purpose of this study was to describe circumstances surrounding adrenal crisis events in children with CAH; to explore parents' perceptions of the consequences of having a child with a life-threatening condition; and to examine a relationship between parents' perceived management ability and the impact CAH has on the family. In Phase 1 of this mixed-methods, cross-sectional study, 77 parents were asked to complete questionnaires comprising measures of family life in the context of childhood illness. Descriptive statistics were computed with four separate analyses using linear mixed models allowing for correlation between responses from parents of the same family and for the variance to be different for fathers and mothers. The following relationships were examined: (1) parental management ability and type of provider instruction on how to manage adrenal crises; (2) parental management ability and child age; (3) the perceived impact of the condition on the family and management ability; and (4) the age of the child and number of adrenal crisis events. In Phase 2, 16 semi-structured interviews were conducted to elicit detailed descriptions of parents' experiences in managing crises. There was a significant, positive relationship between detailed provider instruction to parents on adrenal crisis management and perceived management ability (p = .02), additionally the stronger the perceived management ability, the less impact CAH had on the family (p crisis events and less perceived ability to manage the condition when compared with parents of older children (p = .009). The threat of an adrenal crisis event is a pervasive concern for parents, especially when they are not being properly prepared by providers. Provider support is needed for these parents throughout childhood, but especially in the first 5 years of

  19. Magnetic Resonance Imaging of the Vocal Folds in Women with Congenital Adrenal Hyperplasia and Virilized Voices

    Science.gov (United States)

    Nygren, Ulrika; Isberg, Bengt; Arver, Stefan; Hertegård, Stellan; Södersten, Maria; Nordenskjöld, Agneta

    2016-01-01

    Purpose: Women with congenital adrenal hyperplasia (CAH) may develop a virilized voice due to late diagnosis or suboptimal suppression of adrenal androgens. Changes in the vocal folds due to virilization have not been studied in vivo. The purpose was to investigate if the thyroarytenoid (TA) muscle is affected by virilization and correlate…

  20. Congenital adrenal hyperplasia and brain magnetic resonance imaging abnormalities.

    Science.gov (United States)

    Samia, Younes-Mhenni; Mahdi, Kamoun; Baha, Zantour; Saida, Jerbi-Ommezine; Tahar, Sfar Mohamed; Habib, Sfar Mohamed

    2010-10-01

    A 15-yr-old male patient with congenital adrenal hyperplasia (CAH) was referred to our department with a one year history of gradual worsening of tremors. He was diagnosed with salt-wasting 21-hydroxylase deficiency CAH at 40 d old and was started on hydrocortisone, fludrocortisone and salt. He was found to have hypertension at 8 yr of age. Detailed investigations failed to detect any cause for secondary hypertension. Physical findings on the current hospitalization objectified obesity, blood pressure of 150/80 mmHg, postural and action tremor, left cerebellar syndrome, reflex tetra pyramidal syndrome and mental decline. Brain magnetic resonance imaging (MRI) showed bilateral periventricular white matter hyperintensity that was more pronounced in the posterior regions and associated with cortico-subcortical atrophy and complete agenesis of the corpus callosum. All investigations for leukoencephalopathy were negative. A diagnosis of brain MRI abnormalities related to CAH was made, and the patient received symptomatic treatment of tremors. Our case report provides evidence of an increased frequency of brain MRI abnormalities in CAH. The literature suggests hormonal imbalance and exposure to excess exogenous glucocorticoids as main probable mechanisms. Thus, in clinical practice, CAH should be considered as one of the possible causes of brain white matter involvement associated with or without cerebral atrophy.

  1. Communication about Congenital Adrenal Hyperplasia: Perspective of Filipino Families.

    Science.gov (United States)

    Abad, Peter James B; Anonuevo, Cora A; Daack-Hirsch, Sandra; Abad, Lorna R; Padilla, Carmencita D; Laurino, Mercy Y

    2017-08-01

    Congenital adrenal hyperplasia (CAH), like other genetic conditions, is a relational disease from both the biological and psychosocial perspectives since the diagnosis gives rise to a variety of health, reproductive, and psychosocial implications. It is in these contexts that family communication of genetic information is important to study. Hence, this research aimed to explore genetic information communication in Filipino families affected with CAH. Using a qualitative descriptive design, families with a child affected with CAH were recruited through the CAH parent support group and were interviewed. Semi-structured interviews explored flow and content of genetic information communicated, the meanings the families attach to the communicated information, and the motivating and hindering factors in communication. Thematic analysis was used to analyze the findings. A total of five families participated, which included 11 individuals. Findings revealed that the diagnosis of CAH is not kept secret and it is openly shared with the family. The decision to communicate is influenced by several factors including the family's desire to seek further information about their family history. Initially, the focus of the communicated information is on the health implications and while communication about genetics occurs, this is almost always confined to the immediate family. The mother and grandmother serve as primary communicators in the family. The families have limited understanding of CAH especially its genetic implications including recurrence risk and carrier status. The findings can guide genetic counselors in supporting families in communicating information about CAH with the rest of the family.

  2. Congenital Adrenal Hyperplasia: Classification of Studies Employing Psychological Endpoints

    Directory of Open Access Journals (Sweden)

    Sandberg DavidE

    2010-08-01

    Full Text Available Psychological outcomes in persons with congenital adrenal hyperplasia (CAH have received substantial attention. The objectives of this paper were to (1 catalog psychological endpoints assessed in CAH outcome studies and (2 classify the conceptual/theoretical model shaping the research design and interpretation of CAH-related psychological effects. A total of 98 original research studies, published between 1955 and 2009, were categorized based on psychological endpoints examined as well as the research design and conceptual model guiding analysis and interpretation of data. The majority of studies (68% investigated endpoints related to psychosexual differentiation. The preponderance of studies (76% examined a direct relationship (i.e., inferring causality between prenatal androgen exposure and psychological outcomes. Findings are discussed in relation to the observed imbalance between theoretical interest in the role of prenatal androgens in shaping psychosexual differentiation and a broader conceptual model that examines the role of other potential factors in mediating or moderating the influence of CAH pathophysiology on psychological outcomes in both affected females and males. The latter approach offers to identify factors amenable to clinical intervention that enhance both health and quality of life outcomes in CAH as well as other disorders of sex development.

  3. Testicular adrenal rest tumor in infertile man with congenital adrenal hyperplasia: case report and literature review

    Directory of Open Access Journals (Sweden)

    Giovanni Scala Marchini

    Full Text Available CONTEXT: Synthesis of cortisol and aldosterone is impaired in patients with congenital adrenal hyperplasia (CAH because of 21-hydroxylase deficiency. Men with CAH have low fertility rates compared with the normal population, and this is related to testicular adrenal rest tumors. Findings of azoospermia in combination with a testicular tumor on ultrasound are likely to have a mechanical cause, especially when in the testicular mediastinum. The preferred treatment method consists of intensive corticoid therapy. However, when the tumor is unresponsive to steroid therapy, surgical treatment should be considered. CASE REPORT: We present the case of a male patient with CAH due to 21-hydroxylase deficiency who presented a testicular tumor and azoospermia. Treatment with low daily corticoid doses had previously been started by an endocrinologist, but after 12 months, no significant change in sperm count was found. Although the adrenocorticotrophic hormone and 17-hydroxyprogesterone levels returned to normal values, the follicle-stimulating hormone (FSH, luteinizing hormone and testosterone levels remained unchanged. Ultrasound examination confirmed that the testicles were small and heterogenous bilaterally, and revealed a mosaic area at the projection of the testis network bilaterally. Magnetic resonance imaging confirmed the finding. Testicular biopsy revealed the presence of preserved spermatogenesis and spermiogenesis in 20% of the seminiferous tubules in the right testicle. The patient underwent testis-sparing tumor resection. After 12 months of follow-up, there was no tumor recurrence but the patient still presented azoospermia and joined an intracytoplasmic sperm injection program.

  4. Gender Identity in Patients with Congenital Adrenal Hyperplasia.

    Science.gov (United States)

    Razzaghy-Azar, Maryam; Karimi, Sakineh; Shirazi, Elham

    2017-07-01

    Sex assignment in infancy for patients with disorder of sex development (DSD) is a challenging problem. Some of the patients with congenital adrenal hyperplasia (CAH) have DSD that may affect their gender identity. The study aimed to assess gender identity in patients with CAH. In this study, 52 patients with CAH, including 22 prepubertal children and 30 adolescents and adults, were assessed using two separate gender identity questionnaires for children and adults based on the criteria of diagnostic and statistical manual of mental disorders, 5th edition. In the children group, compatibility was seen between gender identity and rearing gender. In the adult group, there were three cases of mismatching between gender identity and sex assignment composed of two females with poor control and one male with good control with 21-hydroxylase deficiency (21-OHD). Three girls with 11-hydroxylase deficiency (11-OHD) were reared as boy. Two of them with late diagnosis at 5 and 6 years of age had pseudoprecocious puberty. Parents and children did not accept to change the gender. One of them is 36 years old now, is depressed and unsatisfied with her gender, another girl is still child and has male sexual identity. One girl with 11-OHD and early diagnosis at birth with Prader 5 virilization but with good hormonal control was changed to female gender at 12 years of age when female sexual characteristics appeared; she is 34-years-old now, married, and with two children, and she is satisfied with her gender. In patients with CAH, gender identity disorder is a rare finding. Hormonal control, social, familial, and religious beliefs have impacts on gender identity of these patients.

  5. Leydig Cell Tumor Associated with Testicular Adrenal Rest Tumors in a Patient with Congenital Adrenal Hyperplasia due to 11β-Hydroxylase Deficiency

    OpenAIRE

    Charfi, Nadia; Kamoun, Mahdi; Feki Mnif, Mouna; Mseddi, Neila; Mnif, Fatma; Kallel, Nozha; Ben Naceur, Basma; Rekik, Nabila; Fourati, Hela; Daoud, Emna; Mnif, Zainab; Hadj Sliman, Mourad; Sellami-Boudawara, Tahia; Abid, Mohamed

    2012-01-01

    Congenital adrenal hyperplasia (CAH) describes a group of inherited autosomal recessive disorders characterized by enzyme defects in the steroidogenic pathways that lead to the biosynthesis of cortisol, aldosterone, and androgens. Chronic excessive adrenocorticotropic hormone (ACTH) stimulation may result in hyperplasia of ACTH-sensitive tissues in adrenal glands and other sites such as the testes, causing testicular masses known as testicular adrenal rest tumors (TARTs). Leydig cell tumors (...

  6. Clinical manifestations of testicular adrenal rest tumor in males with congenital adrenal hyperplasia

    Directory of Open Access Journals (Sweden)

    Min Kyung Yu

    2015-09-01

    Full Text Available PurposeIn male patients with congenital adrenal hyperplasia (CAH, the presence of testicular adrenal rest tumors (TARTs have been reported, however their prevalence and clinical manifestations are not well known. Untreated TARTs may lead to testicular structural damage and infertility. This study was conducted to investigate the prevalence of TARTs in male patients with CAH, and characterize the manifestations to identify contributing factors to TART.MethodsAmong 102 CAH patients aged 0-30 years, 24 male patients have been regularly followed up in our outpatient clinic at Severance Children's Hospital from January 2000 to December 2014. In order to reveiw the characteristics of TART patients, we calculated the mean levels of hormones during the 5 years before the time of investigation. Five patients underwent follow-up scrotal ultrasonography (US after adjusting the dosage of glucocorticoids.ResultsTARTs were detected in 8 of the 13 patients (61.5%. The median age of TARTs diagnosis was 20.2 years with the youngest case being 15.5 years old. The mean serum level of adrenocorticotropic hormone (ACTH was higher in the TARTs patient group compared to the non-TARTs group (P<0.05. The tumor size decreased in 3 cases, slightly increased in 1 case, and had no change in another case.ConclusionThe serum ACTH level might be associated with the growth promoting factor for TARTs, but the exact mechanism has not been clearly identified. Screening for TARTs using US is important in male patients with CAH for early-detection and prevention of ongoing complications, such as infertility.

  7. Physiological Basis for the Etiology, Diagnosis, and Treatment of Adrenal Disorders: Cushing’s Syndrome, Adrenal Insufficiency, and Congenital Adrenal Hyperplasia

    Science.gov (United States)

    Raff, Hershel; Sharma, Susmeeta T.; Nieman, Lynnette K.

    2014-01-01

    The hypothalamic-pituitary-adrenal (HPA) axis is a classic neuroendocrine system. One of the best ways to understand the HPA axis is to appreciate its dynamics in the variety of diseases and syndromes that affect it. Excess glucocorticoid activity can be due to endogenous cortisol overproduction (spontaneous Cushing’s syndrome) or exogenous glucocorticoid therapy (iatrogenic Cushing’s syndrome). Endogenous Cushing’s syndrome can be subdivided into ACTH-dependent and ACTH-independent, the latter of which is usually due to autonomous adrenal overproduction. The former can be due to a pituitary corticotroph tumor (usually benign) or ectopic ACTH production from tumors outside the pituitary; both of these tumor types overexpress the proopiomelanocortin gene. The converse of Cushing’s syndrome is the lack of normal cortisol secretion and is usually due to adrenal destruction (primary adrenal insufficiency) or hypopituitarism (secondary adrenal insufficiency). Secondary adrenal insufficiency can also result from a rapid discontinuation of long-term, pharmacological glucocorticoid therapy because of HPA axis suppression and adrenal atrophy. Finally, mutations in the steroidogenic enzymes of the adrenal cortex can lead to congenital adrenal hyperplasia and an increase in precursor steroids, particularly androgens. When present in utero, this can lead to masculinization of a female fetus. An understanding of the dynamics of the HPA axis is necessary to master the diagnosis and differential diagnosis of pituitary-adrenal diseases. Furthermore, understanding the pathophysiology of the HPA axis gives great insight into its normal control. PMID:24715566

  8. The Medical Home Concept and Congenital Adrenal Hyperplasia: a Comfortable Habitat!

    Directory of Open Access Journals (Sweden)

    Witchel SelmaFeldman

    2010-05-01

    Full Text Available Patient-centered interdisciplinary health care for children with chronic medical disorders represents an evolution from the traditional "stop and go" treatment for acute illnesses. This model for health care delivery has been called the "medical home," a concept that was originally developed in pediatrics for the care of children with special needs. Patient and family-centered, comprehensive, interdisciplinary, culturally effective, and readily accessible health care delivery is desirable for the care of children with congenital adrenal hyperplasia. As children with congenital adrenal hyperplasia (CAH become adolescents and young adults, transfer of this health care delivery model to adult endocrinologists is appropriate.

  9. The Medical Home Concept and Congenital Adrenal Hyperplasia: A Comfortable Habitat!

    Directory of Open Access Journals (Sweden)

    Selma Feldman Witchel

    2010-01-01

    Full Text Available Patient-centered interdisciplinary health care for children with chronic medical disorders represents an evolution from the traditional “stop and go” treatment for acute illnesses. This model for health care delivery has been called the “medical home,” a concept that was originally developed in pediatrics for the care of children with special needs. Patient and family-centered, comprehensive, interdisciplinary, culturally effective, and readily accessible health care delivery is desirable for the care of children with congenital adrenal hyperplasia. As children with congenital adrenal hyperplasia (CAH become adolescents and young adults, transfer of this health care delivery model to adult endocrinologists is appropriate.

  10. Alterations in Hydrocortisone Pharmacokinetics in a Patient With Congenital Adrenal Hyperplasia Following Bariatric Surgery

    OpenAIRE

    Mallappa, Ashwini; Nella, Aikaterini A.; Kumar, Parag; Brooks, Kristina M.; Perritt, Ashley F.; Ling, Alexander; Liu, Chia-Ying; Merke, Deborah P.

    2017-01-01

    Management of adult patients with classic congenital adrenal hyperplasia (CAH) is challenging and often complicated by obesity, metabolic syndrome, and adverse cardiovascular risk. Alterations in weight can influence cortisol kinetics. A 19-year-old woman with classic CAH and morbid obesity experienced persistent elevations of androgen levels while receiving oral glucocorticoid therapy. Control of adrenal androgens was improved with continuous subcutaneous hydrocortisone infusion therapy, but...

  11. What Are the Treatments for Congenital Adrenal Hyperplasia (CAH)?

    Science.gov (United States)

    ... Español NICHD Theme Browse AZTopics Browse A-Z Adrenal Gland Disorders Autism Spectrum Disorder (ASD) Down Syndrome Endometriosis Learning ... Funding Opportunities & Notices Health A to Z List Adrenal Gland Disorders About NICHD Research Information Find a Study More ...

  12. Fetal sex determination from maternal plasma in pregnancies at risk for congenital adrenal hyperplasia

    NARCIS (Netherlands)

    Rijnders, R. J.; van der Schoot, C. E.; Bossers, B.; de Vroede, M. A.; Christiaens, G. C.

    2001-01-01

    To determine first-trimester fetal sex by isolating free fetal DNA from maternal plasma. The index case was a pregnant woman who previously delivered a girl with congenital adrenal hyperplasia. The SRY gene as a marker for the fetal Y chromosome was detected in maternal serum and plasma by

  13. Cardiovascular and metabolic risk in pediatric patients with congenital adrenal hyperplasia due to 21 hydroxylase deficiency

    NARCIS (Netherlands)

    Mooij, C.F.; Herwaarden, A.E. van; Sweep, F.C.; Roeleveld, N.; Korte, C.L. de; Kapusta, L.; Claahsen-van der Grinten, H.L.

    2017-01-01

    BACKGROUND: The aim of the study was to evaluate the cardiovascular and metabolic risk profile in pediatric patients with congenital adrenal hyperplasia (CAH). METHODS: A cross-sectional study was performed in 27 CAH patients (8-16 years). Blood samples were taken to evaluate circulating

  14. Newborn screening for congenital adrenal hyperplasia in Tokyo, Japan from 1989 to 2013: a retrospective population-based study.

    Science.gov (United States)

    Tsuji, Atsumi; Konishi, Kaoru; Hasegawa, Satomi; Anazawa, Akira; Onishi, Toshikazu; Ono, Makoto; Morio, Tomohiro; Kitagawa, Teruo; Kashimada, Kenichi

    2015-12-15

    Congenital adrenal hyperplasia (CAH) cause life-threatening adrenal crisis. It also affects fetal sex development and can result in incorrect sex assignment at birth. In 1989, a newborn screening program for congenital adrenal hyperplasia (CAH) was introduced in Tokyo. Here we present the results of this screening program in order to clarify the efficiency of CAH screening and the incidence of CAH in Japan. From 1989 to 2013, a total of 2,105,108 infants were screened for CAH. The cutoff level for diagnosis of CAH was adjusted for gestational age and birth weight. A total of 410 infants were judged positive, and of these, 106 patients were diagnosed with CAH, indicating a positive predictive value (PPV) of 25.8 %. Of the 106 patients, 94 (88.7 %) were diagnosed with 21-OHD. Of these 94 patients, 73 were diagnosed with the salt wasting form, 14 with the simple virilising form and 7 with the nonclassical form (NC21OHD). The mean birth weight and gestational age were 3192 ± 385 g and 38.9 ± 1.38 weeks. 11 out of 44 female patients were assigned as female according to their screening result. These data suggest that the newborn screening in Tokyo was effective, especially for sex assignment and preventing fatal adrenal crisis. The incidence of CAH was similar to that measured in previous Japanese screening studies, and it was also similar to that of western countries. The incidence of NC21OHD in Japan in the present study was lower than that in western countries as previous studies reported. The screening program achieved higher PPV than previous CAH screening studies, which might be due to the use of variable cutoffs according to gestational age and birth weight. However, most of the neonates born at 37 weeks or less that were referred to hospital were false-positives. Further changes are needed to reduce the number of false positive preterm neonates.

  15. Multiple osteoblastomas in a child with Cushing syndrome due to bilateral adrenal micronodular hyperplasias

    Directory of Open Access Journals (Sweden)

    Hyeoh Won Yu

    2016-03-01

    Full Text Available Adrenocorticotropin-independent adrenal hyperplasias are rare diseases, which are classified into macronodular (>1 cm and micronodular (≤1 cm hyperplasia. Micronodular adrenal hyperplasia is subdivided into primary pigmented adrenocortical disease and a limited or nonpigmented form 'micronodular adrenocortical disease (MAD', although considerable morphological and genetic overlap is observed between the 2 groups. We present an unusual case of a 44-month-old girl who was diagnosed with Cushing syndrome due to MAD. She had presented with spotty pigmentation on her oral mucosa, lips and conjunctivae and was diagnosed with multiple bone tumors in her femur, pelvis and skull base at the age of 8 years. Her bone tumor biopsies were compatible with osteoblastoma. This case highlights the importance of verifying the clinicopathologic correlation in Cushing syndrome and careful follow-up and screening for associated diseases.

  16. Diabetic fetopathy associated with bilateral adrenal hyperplasia and ambiguous genitalia: a case report

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    Tantbirojn Patou

    2008-07-01

    Full Text Available Abstract Introduction Many fetal malformations can occur because of maternal diabetes. However, ambiguous genital organs have never been reported as an associated finding in the literature. This is the first report of associated ambiguous genital organ and bilateral adrenal hyperplasia in a case of diabetic fetopathy. Case presentation A 19-year-old Thai primigravida with familial history of diabetes mellitus (DM was diagnosed as having gestational DM type 2, based on 100 g oral glucose tolerance test, and was poorly controlled with insulin injections. Delayed targeted ultrasonography at 28 weeks gestation revealed multiple fetal anomalies. The woman underwent low transverse cesarean section at 30 weeks gestation due to preterm labor and transverse lie. The newborn with ambiguous genitalia was delivered but expired after birth. Autopsy findings revealed alobar holoprosencephaly, a prominent forehead, hypotelorism, an absent nose, absent bilateral ears, median cleft lip and palate, preaxial polydactyly of the right hand, accessory spleens, single umbilical artery, markedly enlarged adrenal glands and ambiguous external genitalia The subsequent fetal chromosomal study revealed 46,XX. Conclusion We describe a case of diabetic fetopathy with classic facial malformation and preaxial hallucal polydactyly which has been proposed as a marker of diabetic embryopathy. Bilateral adrenal hyperplasia with ambiguous genitalia, an uncommon associated anomaly, was also identified. It is controversial whether adrenal hyperplasia can be a novel feature of diabetic fetopathy or just a coincidental finding. Further observation and adequate investigation are needed in such cases.

  17. Cell-to-cell communication in bilateral macronodular adrenal hyperplasia causing hypercortisolism

    Directory of Open Access Journals (Sweden)

    Herve eLefebvre

    2015-04-01

    Full Text Available It has been well established that, in the human adrenal gland, cortisol secretion is not only controlled by circulating corticotropin but is also influenced by a wide variety of bioactive signals, including conventional neurotransmitters and neuropeptides, released within the cortex by various cell types such as chromaffin cells, neurons, cells of the immune system, adipocytes and endothelial cells. These different types of cells are present in bilateral macronodular adrenal hyperplasia, a rare etiology of primary adrenal Cushing’s syndrome, where they appear intermingled with adrenocortical cells in the hyperplastic cortex. In addition, the genetic events which cause the disease favor abnormal adrenal differenciation that results in illicit expression of paracrine regulatory factors and their receptors in adrenocortical cells. All these defects constitute the molecular basis for aberrant autocrine/paracrine regulatory mechanisms which are likely to play a role in the pathophysiology of bilateral macronodular adrenal hyperplasia-associated hypercortisolism. The present review summarizes the current knowledge on this topic as well as the therapeutic perspectives offered by this new pathophysiological concept.

  18. Failure to visualize adrenal glands in a patient with bilateral adrenal hyperplasia

    International Nuclear Information System (INIS)

    Gordon, L.; Mayfield, R.K.; Levine, J.H.; Lopes-Virella, M.F.; Sagel, J.; Buse, M.G.

    1980-01-01

    A patient with clinical and biochemical evidence of Cushing's disease and severe hyperlipidemia underwent an adrenal imaging procedure with NP-59 (6β-[ 131 I]iodomethyl-19-norcholesterol), without visualization of either gland. Correction of the hyperlipidemia followed by repeated adrenal imaging resulted in bilateral visualization. A pituitary tumor was removed at surgery, confirming the diagnosis of Cushing's disease

  19. First Case of Bacteremia Due to Kytococcus schroeteri in a Child with Congenital Adrenal Hyperplasia.

    Science.gov (United States)

    Bayraktar, Banu; Dalgic, Nazan; Duman, Nurcan; Petmezci, Ercüment

    2018-03-22

    Kytococcus schroeteri is a commensal organism of the human skin, may cause serious infections in immunocompromised patients. We report the first case of Kytococcus schroeteri bacteremia in a child with congenital adrenal hyperplasia (CAH), identified by matrix-assisted laser desorption ionization-time of flight mass spectrometry (MALDI-TOF MS)(Bruker Daltonics, Germany). İdentification was confirmed by 16S rRNA gene sequencing.

  20. The Role of gsp Mutations on the Development of Adrenocortical Tumors and Adrenal Hyperplasia.

    Science.gov (United States)

    Villares Fragoso, Maria Candida Barisson; Wanichi, Ingrid Quevedo; Cavalcante, Isadora Pontes; Mariani, Beatriz Marinho de Paula

    2016-01-01

    Somatic GNAS point mutations, commonly known as gsp mutations, are involved in the pathogenesis of McCune-Albright syndrome (MAS) and have also been described in autonomous hormone-producing tumors, such as somatotropinoma, corticotrophoma, thyroid cancer, ovarian and testicular Leydig cell tumors, and primary macronodular adrenocortical hyperplasia (PMAH) (1-3). The involvement of gsp mutations in adrenal tumors was first described by Lyons et al. Since then, several studies have detected the presence of gsp mutations in adrenal tumors, but none of them could explain its presence along or the mechanism that leads to tumor formation and hormone hypersecretion. As a result, the molecular pathogenesis of the majority of sporadic adrenocortical tumors remains unclear (3). PMAH has also been reported with gsp somatic mutations in a few cases. Fragoso et al. identified two distinct gsp somatic mutations affecting arginine residues on codon 201 of GNAS in a few patients with PMAH who lacked any features or manifestations of MAS. Followed by this discovery, other studies have continued looking for gsp mutations based on strong prior evidence demonstrating that increased cAMP signaling is sufficient for cell proliferation and cortisol production (2, 4). With consideration for the previously reported findings, we conjecture that although somatic activating mutations in GNAS are a rare molecular event, these mutations could probably be sufficient to induce the development of macronodule hyperplasia and variable cortisol secretion. In this manuscript, we revised the presence of gsp mutations associated with adrenal cortical tumors and hyperplasia.

  1. Unilateral testicular tumour associated to congenital adrenal hyperplasia: Failure of specific tumoral molecular markers to discriminate between adrenal rest and leydigioma.

    Science.gov (United States)

    Fenichel, P; Bstandig, B; Roger, C; Chevallier, D; Michels, J-F; Sadoul, J-L; Hieronimus, S; Brucker-Davis, F

    2008-11-01

    Testicular adrenal rest tumours are frequently associated with congenital adrenal hyperplasia (CAH). These ACTH-dependent tumours cannot be easily distinguished histologically from Leydig-cell tumours. We report the case of a 30-year-old man who was explored for infertility, azoospermia and unilateral testicular tumour. High levels of 17-OH progesterone and ACTH, low cortisol and undetectable gonadotropins levels, associated to bilateral adrenal hyperplasia, led to the diagnosis of CAH by 21-OH deficiency with a composite heterozygoty. The testicular tumour was first considered as adrenal rest. However, histological analysis of this unilateral painful tumour showed a steroid-hormone-secreting cell proliferation with atypical and frequent mitosis. To discriminate between a benign adrenal rest tumour and a possible malignant leydigioma, tumoral expression of specific gene products was analyzed by RT-PCR. No 11-beta-hydroxylase nor ACTH receptor mRNAs could be found in the tumour, which did not behave like usual adrenal rest cells. For this unilateral testicular tumour, the lack of adrenal-specific markers associated with a high rate of mitosis and pleiomorphism supported a leydigian origin with malignant potential. However, lack of tumoral LH-R mRNA expression and a tumour-free 3-year follow-up led us to retain the diagnosis of adrenal rest tumour with loss of adrenal gene expression and progressive autonomous behaviour.

  2. Gendered Peer Involvement in Girls with Congenital Adrenal Hyperplasia: Effects of Prenatal Androgens, Gendered Activities, and Gender Cognitions.

    Science.gov (United States)

    Berenbaum, Sheri A; Beltz, Adriene M; Bryk, Kristina; McHale, Susan

    2018-01-09

    A key question in understanding gender development concerns the origins of sex segregation. Children's tendencies to interact with same-sex others have been hypothesized to result from gender identity and cognitions, behavioral compatibility, and personal characteristics. We examined whether prenatal androgen exposure was related to time spent with boys and girls, and how that gendered peer involvement was related to sex-typed activities and gender identity and cognitions. We studied 54 girls with congenital adrenal hyperplasia (CAH) aged 10-13 years varying in degree of prenatal androgen exposure: 40 girls with classical CAH (C-CAH) exposed to high prenatal androgens and 14 girls with non-classical CAH (NC-CAH) exposed to low, female-typical, prenatal androgens. Home interviews and questionnaires provided assessments of gendered activity interests and participation, gender identity, and gender cognitions. Daily phone calls over 7 days assessed time spent in gendered activities and with peers. Girls with both C-CAH and NC-CAH interacted more with girls than with boys, with no significant group differences. The groups did not differ significantly in gender identity or gender cognitions, but girls with C-CAH spent more time in male-typed activities and less time in female-typed activities than did girls with NC-CAH. Time spent with girls reflected direct effects of gender identity/cognitions and gender-typed activities, and an indirect effect of prenatal androgens (CAH type) through gender-typed activities. Our results extend findings that prenatal androgens differentially affect gendered characteristics and that gendered peer interactions reflect combined effects of behavioral compatibility and feelings and cognitions about gender. The study also shows the value of natural experiments for testing hypotheses about gender development.

  3. Growth of a progesterone receptor-positive meningioma in a female patient with congenital adrenal hyperplasia.

    Science.gov (United States)

    O'Shea, T; Crowley, R K; Farrell, M; MacNally, S; Govender, P; Feeney, J; Gibney, J; Sherlock, M

    2016-01-01

    Meningioma growth has been previously described in patients receiving oestrogen/progestogen therapy. We describe the clinical, radiological, biochemical and pathologic findings in a 45-year-old woman with congenital adrenal hyperplasia secondary to a defect in the 21-hydroxylase enzyme who had chronic poor adherence to glucocorticoid therapy with consequent virilisation. The patient presented with a frontal headache and marked right-sided proptosis. Laboratory findings demonstrated androgen excess with a testosterone of 18.1 nmol/L (0-1.5 nmol) and 17-Hydroxyprogesterone >180 nmol/L (transsexual patients undergoing therapy with high-dose oestrogen and progestogens. Progesterone receptor positivity has been described previously in meningiomas. 17-Hydroxyprogesterone is elevated in CAH and has affinity and biological activity at the progesterone receptor. Therefore, we hypothesise that patients who have long-standing increased adrenal androgen precursor concentrations may be at risk of meningioma growth. Patients with long-standing CAH (particularly if not optimally controlled) may present with other complications, which may be related to long-standing elevated androgen or decreased glucocorticoid levels.Chronic poor control of CAH is associated with adrenal myelolipoma and adrenal rest tissue tumours.Meningiomas are sensitive to endocrine stimuli including progesterone, oestrogen and androgens as they express the relevant receptors.

  4. Testicular adrenal rest tumours in boys, adolescents and adult men with congenital adrenal hyperplasia may be associated with the CYP21A2 mutation

    DEFF Research Database (Denmark)

    Mouritsen, Annette; Jørgensen, Niels; Main, Katharina M

    2010-01-01

    % of CAH adults and may already appear during childhood. Whether genotype sub-types can account for the development of TART has not been investigated previously. We therefore investigated this by coupling clinical information of CAH patients with information of their genetic mutation. In 49 male patients......Congenital adrenal hyperplasia (CAH) is an autosomal recessive disorder with impaired function of the adrenal cortex caused by mutations in the CYP21A2 gene. Deficiency of steroid 21-hydroxylase accounts for 80-95% of CAH cases. Testicular adrenal rest tumours (TART) may be prevalent in up to 95...

  5. Alteraciones neuropsicológicas en la hiperplasia adrenal congénita Neuropsychological disorders related to congenital adrenal hyperplasia

    Directory of Open Access Journals (Sweden)

    Yaser Ramírez Benítez

    2008-12-01

    Full Text Available INTRODUCCIÓN: la hiperplasia adrenal congénita engloba un conjunto de enfermedades genéticas de transmisión autosómica recesiva caracterizadas por un trastorno de la esteroidogénesis suprarrenal. Constituye un caso típico de daño cerebral por el efecto patológico de las hormonas sexuales en el sistema nervioso en desarrollo, y a esto se le suman las secuelas secundarias al cuadro grave de crisis adrenal que aparece en la forma clásica a los pocos días de vida. MÉTODOS: estudio de caso en un niño de 6 años de edad con antecedentes de hiperplasia adrenal congénita perdedora de sal. Se determinaron los puntos débiles y fuertes en el desarrollo a través de la batería neuropsicológica Luria Inicial. Como complementario a la evaluación se aplicó la Escala de Inteligencia de Weschler y el Cuestionario de Comportamiento Infantil para padres y profesores. RESULTADOS: el niño presenta un perfil neuropsicológico caracterizado por bajas puntuaciones en: motricidad manual, habilidades viso-espaciales, impulsividad, pobre vocabulario, dificultades en operaciones de cálculos sencillos, en el reconocimiento de objetos por el tacto sin la ayuda visual y en la rapidez de procesamiento. La capacidad de inteligencia está en límites normales con un rendimiento más bajo en la escala no verbal. En la escala de comportamiento se identificaron las dificultades en la atención y en el aprendizaje. CONCLUSIONES: el perfil neuropsicológico se caracteriza por tener bajas ejecuciones en tareas verbales, no verbales y en la velocidad de procesamiento. El tratamiento hormonal en los primeros días de nacido permite que el desarrollo siga un curso "normal", pero todo proceso tiene un costo que se refleja en la adquisición de habilidades psicológicas superioresCongenital adrenal hyperplasia includes a series of genetic diseases of autosomal recessive transmission characterized by a disorder of suprarenal esteroidogenesis. This is a typical case of

  6. Aberrant expression of glucagon receptors in adrenal glands of a patient with Cushing's syndrome and ACTH-independent macronodular adrenal hyperplasia

    Directory of Open Access Journals (Sweden)

    Valeria de Miguel

    2010-06-01

    Full Text Available Adrenocorticotropin (ACTH independent bilateral macronodular adrenal hyperplasia (AIMAH is a rare cause of Cushing´s syndrome, characterized by bilateral adrenal lesions and excess cortisol production despite ACTH suppression. Cortisol synthesis is produced in response to abnormal activation of G-protein- coupled receptors, such as gastric inhibitory peptide, vasopressin, beta adrenergic agonists, LH/hCG and serotonin receptors. The aim of this study was to analyze the expression of glucagon receptors in adrenal glands from an AIMAH patient. A patient with ACTH-independent Cushing´s syndrome and bilateral macronodular adrenal hyperplasia was screened for altered activation of adrenal receptors by physiological (mixed meal and pharmacological (gonadotrophin releasing hormone, ACTH and glucagon tests. The results showed abnormally high levels of serum cortisol after stimulation with glucagon. Hypercortisolism was successfully managed with ketoconazole treatment. Interestingly, a 4-month treatment with a somatostatin analogue (octreotide was also able to reduce cortisol secretion. Finally, Cushing's syndrome was cured after bilateral adrenalectomy. Abnormal mRNA expression for glucagon receptor in the patient´s adrenal glands was observed by Real-Time PCR procedure. These results strongly suggest that the mechanism of AIMAH causing Cushing´s syndrome in this case involves the illicit activation of adrenal glucagon receptors. This is the first case reported of AIMAH associated with ectopic glucagon receptors.

  7. High corticosterone and sex reversal in common carp (Cyprinus carpio L.) with adrenal hyperplasia caused by P450c17a2 deficiency

    NARCIS (Netherlands)

    Nematollahi, M.A.; Pelt-Heerschap, van H.M.L.; Komen, H.

    2014-01-01

    The aim of this study was to investigate the inheritance of adrenal hyperplasia, caused by 17a-hydroxylase deficiency, and its association with female to male sex reversal in common carp. Inbred strains used in this experiment were E4 (XX, female, normal), E5 (XX, male, adrenal hyperplasia) and E7

  8. Leydig Cell Tumor Associated with Testicular Adrenal Rest Tumors in a Patient with Congenital Adrenal Hyperplasia due to 11β-Hydroxylase Deficiency

    Directory of Open Access Journals (Sweden)

    Nadia Charfi

    2012-01-01

    Full Text Available Congenital adrenal hyperplasia (CAH describes a group of inherited autosomal recessive disorders characterized by enzyme defects in the steroidogenic pathways that lead to the biosynthesis of cortisol, aldosterone, and androgens. Chronic excessive adrenocorticotropic hormone (ACTH stimulation may result in hyperplasia of ACTH-sensitive tissues in adrenal glands and other sites such as the testes, causing testicular masses known as testicular adrenal rest tumors (TARTs. Leydig cell tumors (LCTs are make up a very small number of all testicular tumors and can be difficult to distinguish from TARTs. This distinction is interesting because LCTs and TARTs require different therapeutic approaches. Hereby, we present an unusual case of a 19-year-old patient with CAH due to 11β-hydroxylase deficiency, who presented with TARTs and an epididymal Leydig cell tumor.

  9. 150 years of congenital adrenal hyperplasia: translation and commentary of De Crecchio's classic paper from 1865.

    Science.gov (United States)

    Delle Piane, Luisa; Rinaudo, Paolo F; Miller, Walter L

    2015-04-01

    In 1865, Luigi De Crecchio, a Neapolitan pathologist, published a detailed autopsy description of Giuseppe Marzo, who lived as a man, had nearly-normal appearing male external genitalia, female internal reproductive organs, and massively enlarged adrenals. This report is widely cited as the first report of non-salt-losing congenital adrenal hyperplasia (CAH), but a complete English translation has not been available. Via interlibrary loan, we obtained the original volume containing De Crecchio's paper. The complete 39-page publication was translated by two reproductive endocrinologists (L.D.P. and P.F.R.) who are native speakers of Italian. A pediatric endocrinologist conversant with CAH (W.L.M.) summarizes and comments on De Crecchio's observations. Anatomically, the external genitalia were characterized by labio-scrotal fusion and a 10-cm curved phallus with hypospadias. Internally, the ovaries, tubes, and uterus were hypoplastic but otherwise normal, except that the uterus inserted into a utricle. The adrenals were massively enlarged, but this observation was dismissed as unimportant. De Crecchio's exposition of Marzo's life shows many of the issues affecting patients today: family ill-ease regarding genital ambiguity at birth, social pressure following reversed sex assignment in childhood, adult embarassment about genital appearance, difficulties with a legal sex assignment on a birth certificate, and substantial efforts to exhibit maleness to self and associates. De Crecchio was an astute observer who provides insight into both nineteenth-century endocrinology and continuing twenty-first-century difficulties in the care of patients with disordered sex development.

  10. Prevalence of ovarian adrenal rest tumours and polycystic ovaries in females with congenital adrenal hyperplasia: results of ultrasonography and MR imaging

    International Nuclear Information System (INIS)

    Stikkelbroeck, Nike M.M.L.; Schouten, Diana; Otten, Barto J.; Hermus, Ad R.M.M.; Suliman, Harold M.; Jager, Gerrit J.; Braat, Didi D.M.

    2004-01-01

    The aim of the investigation was to assess the prevalence of ovarian adrenal rest tumours and polycystic ovaries in female patients with congenital adrenal hyperplasia (CAH). Thirteen female CAH patients (median age 19.8 years, range 14.8-23.5 years) underwent transvaginal (n=6) or transabdominal (n=7) ultrasonography by a gynaecologist and MR imaging (n=13) of the ovaries (pre and post contrast-enhanced T1- and T2-weighted images). Ovarian adrenal rest tumours were defined as small hypoechoic and multifocal nodules on ultrasound and isointense lesions on T1- and hypointense on T2-weighted MR images (derived from characteristics of testicular adrenal rest tumours). Polycystic ovaries were defined as the presence of ≥10 follicles arranged peripherally around or scattered throughout increased stroma. No ovarian adrenal rest tumours were found either on ultrasound, or by MR imaging. Polycystic ovaries were found in 2 of the 13 patients (15.4%), both with ultrasound and MR. No ovarian adrenal rest tumours were detected in these female CAH patients, which suggests that ovarian adrenal rest tumours in CAH females are rare. The prevalence of polycystic ovaries corresponded to that in the general population. From these results, we would suggest that routine ovarian imaging in CAH females is not indicated. However, when ovarian dysfunction is present, ovarian imaging is advised, first by ultrasonography, to detect ovarian adrenal rest tumours or polycystic ovaries. (orig.)

  11. Clinical profile of congenital adrenal hyperplasia and short-term response to treatment

    International Nuclear Information System (INIS)

    Lal, S.; Lal, S.; Jamro, B.U.; Jamro, S.

    2015-01-01

    To determine the clinical presentation of congenital adrenal hyperplasia and short- term response to treatment. Background: Congenital adrenal hyperplasia (CAH) is a genetic disorder which usually presents with life threatening emergencies. Awareness of physicians regarding these presentations is an essential for early diagnosis and lifesaving treatment. In view of the prevalence of the condition as reported from tertiary care centers within the country and other parts of the globe, we had carried out a study in the paediatric department of Shaheed Mohtrama Benazir Bhutto Medical University Larkana. Material and Methods: The study was conducted over a period of one year from July 2012 to June 2013. All infants presenting with vomiting, dehydration, shock, failure to thrive and ambiguous genitalia were examined and investigated thoroughly. The diagnosis was based upon a raised level of serum 17 OHP in a child with suggestive clinical features. Results: A total of 40 children were diagnosed to have CAH during the study period. The major presenting features were vomiting in 13 (32.5%), ambiguous genitalia 17 (42.5%), vomiting and ambiguous genitalia 10 (25%), shock 5(12.5%) and failure to thrive in 13 (32.5%) of cases. All the patients were followed up after initiation of treatment and good response was observed to short-term treatment. Conclusion: This study highlights the importance of common clinical features like vomiting, unreasonable dehydration, shock and ambiguous genitalia being the presenting features of CAH and the effectiveness of replacement therapy in amending life threatening emergencies due to this condition. (author)

  12. Alterations in Hydrocortisone Pharmacokinetics in a Patient With Congenital Adrenal Hyperplasia Following Bariatric Surgery.

    Science.gov (United States)

    Mallappa, Ashwini; Nella, Aikaterini A; Kumar, Parag; Brooks, Kristina M; Perritt, Ashley F; Ling, Alexander; Liu, Chia-Ying; Merke, Deborah P

    2017-07-01

    Management of adult patients with classic congenital adrenal hyperplasia (CAH) is challenging and often complicated by obesity, metabolic syndrome, and adverse cardiovascular risk. Alterations in weight can influence cortisol kinetics. A 19-year-old woman with classic CAH and morbid obesity experienced persistent elevations of androgen levels while receiving oral glucocorticoid therapy. Control of adrenal androgens was improved with continuous subcutaneous hydrocortisone infusion therapy, but obesity-related comorbidities persisted. After undergoing sleeve gastrectomy, the patient experienced dramatic weight loss, with improvement in insulin sensitivity and fatty liver in the postbariatric period. Cortisol clearance studies performed to evaluate changes in hydrocortisone dose requirements showed marked alternations in cortisol pharmacokinetics with decreases in volume of distribution and cortisol clearance, along with an increase in area under the curve for cortisol. Hydrocortisone dose was subsequently decreased 34% by 15 months after surgery. Effective control of androgen excess on this lower hydrocortisone dose was achieved and continues 27 months after surgery. This case highlights obesity-related complications of glucocorticoid replacement therapy in the management of CAH. Individual patient factors, such as fatty liver disease and insulin resistance, can have a clinically important effect on cortisol metabolism. Bariatric surgery was a safe and effective treatment of obesity in this patient with CAH and should be considered for patients with CAH and multiple obesity-related comorbidities.

  13. Magnetic Resonance Imaging of the Vocal Folds in Women With Congenital Adrenal Hyperplasia and Virilized Voices.

    Science.gov (United States)

    Nygren, Ulrika; Isberg, Bengt; Arver, Stefan; Hertegård, Stellan; Södersten, Maria; Nordenskjöld, Agneta

    2016-08-01

    Women with congenital adrenal hyperplasia (CAH) may develop a virilized voice due to late diagnosis or suboptimal suppression of adrenal androgens. Changes in the vocal folds due to virilization have not been studied in vivo. The purpose was to investigate if the thyroarytenoid (TA) muscle is affected by virilization and correlate findings to fundamental frequency (F0). A case-control study using magnetic resonance imaging and voice recordings. Four women with CAH with virilized voices (26-40 years), and 5 female and 4 male controls participated. Measurements of cross-sectional TA muscle area, vocal fold length, vocal tract length, and acoustic analyses of F0 were performed. Women with CAH had larger cross-sectional TA muscle area than female control subjects and smaller than male controls. A significant negative correlation was found between TA muscle area and mean F0. The patients had a smaller physiological voice range than both female and male controls. Data from our small study suggest that a larger TA muscle area is strongly associated with a lower F0 and thus the anatomical explanation for a female virilized voice, suggesting an androgen effect on the vocal folds. The findings from the present study need to be confirmed in a larger study.

  14. Iatrogenic Cushing's Disease in a Boy after Misdiagnosis of Salt-Losing Virilizing Adrenal Hyperplasia: Impaired Metyrapone Response with Failure of Catch-Up Growth

    Science.gov (United States)

    Mendilaharzu, Hernan; And Others

    1973-01-01

    A boy misdiagnosed as having the sodium-losing form of virilizing adrenal hyperplasia was treated with large doses of glucocorticoids and mineralocorticoids from the newborn period until he was more than 4 years of age. (Author)

  15. Congenital adrenal hyperplasia: review from a surgeon´s perspective in the beginning of the 21 st. century

    Directory of Open Access Journals (Sweden)

    LISANDRO ARIEL PIAGGIO

    2014-01-01

    Full Text Available Congenital adrenal hyperplasia (CAH most commonly due to 21-hydroxylase deficiency (21OHD is the most common type of disorder of sex development. This review will focus on CAH addressing historical and current surgical techniques with their anatomical foundations, with special attention to long term results and outcomes on sexual function, patient satisfaction, patient attitude towards surgery, and ongoing controversies in management of these patients.

  16. Urosepsis in infants with vesicoureteral reflux masquerading as the salt-losing type of congenital adrenal hyperplasia

    Energy Technology Data Exchange (ETDEWEB)

    Vaid, Y.N.; Lebowitz, R.L.

    1989-08-01

    Three male infants with vomiting, dehydration, hyponatremia, hyperkalemia and metabolic acidosis were found to have vesicoureteral reflux (VUR) and urinary tract infection. Two were initially thought to have the salt-losing form of congenital adrenal hyperplasia. Although prompt diagnosis of this potentially fatal condition is critical, its mimicry by urosepsis in infants with VUR is actually more common. Infection probably causes unresponsiveness of the distal renal tubules to aldoterone. (orig.).

  17. Unilateral adrenal hyperplasia is a usual cause of primary hyperaldosteronism. Results from a Swedish screening study

    Directory of Open Access Journals (Sweden)

    Sigurjonsdottir Helga

    2012-09-01

    Full Text Available Abstract Background The existence of unilateral adrenal hyperplasia (AH has been considered a rare cause of primary hyperaldosteronism (PA. Methods In a prospective study we screened for PA in a non-selected (NSP and selected hypertensive population (SP, to define the cause of PA. We included 353 consecutive patients with hypertension; age 20 to 88 years, 165 women and 188 men, from a university-based Hypertension and Nephrology Outpatient clinics (123 SP and two primary care centres, (230 NSP from the same catch-up area. Serum aldosterone and plasma renin activity (PRA were measured and the ARR calculated. Verifying diagnostic procedure was performed in patients with both elevated aldosterone and ARR. Patients diagnosed with PA were invited for adrenal venous sampling (AVS and offered laparoscopic adrenalectomy when AVS found the disease to be unilateral. Results After screening, 46 patients, 13% of the whole population (22.8% SP and 7.8% NSP had aldosterone and ARR above the locally defined cut-off limits (0.43 nmol/l and 1.28 respectively. After diagnostic verification, 20 patients (6% had PA, (14.5% SP and 1.4% NSP. Imaging diagnostic procedures with CT-scans and scintigraphy were inconclusive. AVS, performed in 15 patients verified bilateral disease in 4 and unilateral in 10 patients. One AVS failed. After laparoscopic adrenalectomy, 4 patients were found to have adenoma and 5 unilateral AH. One patient denied operation. Conclusion The prevalence of PA was in agreement with previous studies. The study finds unilateral PA common and unilateral AH as half of those cases. As may be suspected PA is found in much higher frequency in specialised hypertensive units compared to primary care centers. AVS was mandatory in diagnosis of unilateral PA.

  18. Newborn screening for congenital adrenal hyperplasia in Cuba: six years of experience.

    Science.gov (United States)

    González, Ernesto Carlos; Carvajal, Frank; Frómeta, Amarilys; Arteaga, Ana Luisa; Castells, Elisa María; Espinosa, Tania; Coto, Remigio; Pérez, Pedro Lucio; Tejeda, Yileidis; Del Río, Lesley; Segura, Mary Triny; Almenares, Pedro; Robaina, René; Fernández, José Luis

    2013-06-05

    Since 2005, a newborn screening program for congenital adrenal hyperplasia (CAH) by measuring 17-alpha-hydroxyprogesterone (17OHP) in dried blood spots was introduced in Cuba. The hormone was measured by the 17OHP Neonatal UMELISA method, in samples collected on the 5th day as average. Confirmatory test was performed to those neonates with 17OHP values above 55 nmol/l. Some perinatal factors that can influence on 17OHP levels were studied. From January 2005 to December 2010, 621,303 newborns were screened and 39 CAH cases were detected. Coverage of the program reached 98%. The incidence of CAH in Cuba was 1:15,931, similar to that reported by other programs. A recall for suspected CAH was performed in 10,799 cases (1.74%). Therapy in classical CAH patients was started at the mean age of 22 days. 17OHP levels were significantly higher in newborns with lower birth-weight (BW) and/or gestational age (GA). In addition, 17OHP values were affected by the gender, twin status or mode of delivery. In Cuba, the nationwide newborn screening program has allowed the early detection of CAH. The use of an optimized cut-off level for BW or GA could lead to a reduction in the percentage of recalled babies. Copyright © 2013 Elsevier B.V. All rights reserved.

  19. LIPOPOLYSACCHARIDE INDUCES THE PRODUCTION OF DIAGNOSTIC MONOCLONAL ANTIBODY BY HYBRIDOMA CELLS AGAINST CONGENITAL ADRENAL HYPERPLASIA

    Directory of Open Access Journals (Sweden)

    GEK KEE CHUA

    2017-11-01

    Full Text Available The purpose of this research is to screen and identify the potential inducers in maximizing the production of monoclonal antibody by hybridoma 192 cell line for Congenital Adrenal Hyperplasia diagnostic. There are nine inducers used in this research, namely lysozyme, aldolase, sodium butyrate, sodium phosphate, potassium phosphate, dimethyl sulfoxide, lipopolysaccharide, essential amino acids, and nonessential amino acids. Hybridoma 192 cell was cultured in 5% CO2 incubator at 37°C and ˃80% humidity in the medium with different concentrations of inducer agents. The inducers were added at the beginning of the culture and the samples were taken after 72 h of culture. The performance of these inducer agents was assessed based on the maximum monoclonal antibody titer achieved using Enzyme-linked Immunosorbent Assay. Lipopolysaccharide was found to increase the maximum monoclonal antibody titer when supplemented at 8 to 12 µg/mL. After optimization using one-factor central composite design at this range, the optimum point was determined to be 8 µg/mL. Verification experiments shows that lipopolysaccharide enhanced the average specific monoclonal antibody production rate by 56% relative to control. In conclusion, lipopolysaccharide at 8 µg/mL is able to increase the monoclonal antibody specific production of hybridoma 192 cell line.

  20. Ehlers-Danlos Syndrome Caused by Biallelic TNXB Variants in Patients with Congenital Adrenal Hyperplasia.

    Science.gov (United States)

    Chen, Wuyan; Perritt, Ashley F; Morissette, Rachel; Dreiling, Jennifer L; Bohn, Markus-Frederik; Mallappa, Ashwini; Xu, Zhi; Quezado, Martha; Merke, Deborah P

    2016-09-01

    Some variants that cause autosomal-recessive congenital adrenal hyperplasia (CAH) also cause hypermobility type Ehlers-Danlos syndrome (EDS) due to the monoallelic presence of a chimera disrupting two flanking genes: CYP21A2, encoding 21-hydroxylase, necessary for cortisol and aldosterone biosynthesis, and TNXB, encoding tenascin-X, an extracellular matrix protein. Two types of CAH tenascin-X (CAH-X) chimeras have been described with a total deletion of CYP21A2 and characteristic TNXB variants. CAH-X CH-1 has a TNXB exon 35 120-bp deletion resulting in haploinsufficiency, and CAH-X CH-2 has a TNXB exon 40 c.12174C>G (p.Cys4058Trp) variant resulting in a dominant-negative effect. We present here three patients with biallelic CAH-X and identify a novel dominant-negative chimera termed CAH-X CH-3. Compared with monoallelic CAH-X, biallelic CAH-X results in a more severe phenotype with skin features characteristic of classical EDS. We present evidence for disrupted tenascin-X function and computational data linking the type of TNXB variant to disease severity. © 2016 WILEY PERIODICALS, INC.

  1. Pathological manifestation of difference in washout pattern of adrenal hyperplasia on dynamic CT

    International Nuclear Information System (INIS)

    Nishie, Akihiro; Asayama, Yoshiki; Ishigami, Kousei

    2014-01-01

    The relationship between the washout pattern and constituent cell in adrenal hyperplasia (AH) has not been fully investigated. The purpose of this study was to elucidate the radiological or pathological factors determining the washout pattern of AH on dynamic CT. Ten patients with 14 surgically proven AHs were enrolled. Dynamic CT was scanned before (pre-contrast image) and 60 seconds (early phase) and 240 seconds (delayed phase) after administration of iodine contrast. The absolute percentage washout (APW) of each nodular lesion was calculated using the following formula: APW(%)=(TAearly-TAdelay) / (TAearly-TApre)×100, when TApre, TAearly and TAdelay were defined as tumour attenuation values of pre-contrast, early and delayed phases, respectively. Pathologically, the clear cell ratio (CCR) constituting each nodular lesion was qualitatively assessed. Regression analysis was performed to evaluate a correlation between each pair of CCR, TApre, (TAearly-TAdelay) and APW. There was a significant correlation between each pair of CCR, TApre and APW. CCR decreased as TApre increased (r=0.81, P<0.001). APW increased as CCR decreased r=0.80, P<0.001) or as TApre increased (r=0.74, P<0.01). The key factors of washout pattern of AH on dynamic CT were CCR and TApre. The difference in constituent cell was associated with variability in APW of AH.

  2. Growth curves for congenital adrenal hyperplasia from a national retrospective cohort.

    Science.gov (United States)

    Bretones, Patricia; Riche, Benjamin; Pichot, Emmanuel; David, Michel; Roy, Pascal; Tardy, Véronique; Kassai, Behrouz; Gaillard, Ségolène; Bernoux, Delphine; Morel, Yves; Chatelain, Pierre; Nicolino, Marc; Cornu, Catherine

    2016-12-01

    In congenital adrenal hyperplasia (CAH), adjusting hydrocortisone dose during childhood avoids reduced adult height. However, there are currently no CAH-specific charts to monitor growth during treatment. Our objective was to elaborate growth reference charts and bone maturation data for CAH patients. We conducted a retrospective observational cohort study, in 34 French CAH centers. Patients were 496 children born 1970-1991 with genetically proven 21-hydroxylase deficiency. Their growth and bone maturation data were collected until age 18 together with adult height, puberty onset, parental height, and treatment. The mean (SD) heights were modeled from birth to adulthood. The median±1 SD and ±2 SDs model-generated curves were compared with the French references. A linear model for bone maturation and a logistic regression model for the probability of short adult height were built. Growth charts were built by sex for salt wasting (SW) and simple virilizing (SV) children treated before 1 year of age. In girls and boys, growth was close to that of the general French population up to puberty onset. There was almost no pubertal spurt and the mean adult height was shorter than that of the general population in girls (-1.2 SD, 156.7 cm) and boys (-1.0 SD, 168.8 cm). Advanced bone age at 8 years had a strong impact on the risk of short adult height (OR: 4.5 per year advance). The 8-year bone age is a strong predictor of adult height. It will help monitoring the growth of CAH-affected children.

  3. Broadening the Spectrum of Ehlers Danlos Syndrome in Patients With Congenital Adrenal Hyperplasia.

    Science.gov (United States)

    Morissette, Rachel; Chen, Wuyan; Perritt, Ashley F; Dreiling, Jennifer L; Arai, Andrew E; Sachdev, Vandana; Hannoush, Hwaida; Mallappa, Ashwini; Xu, Zhi; McDonnell, Nazli B; Quezado, Martha; Merke, Deborah P

    2015-08-01

    The contiguous gene deletion syndrome (CAH-X) was described in a subset (7%) of congenital adrenal hyperplasia (CAH) patients with a TNXA/TNXB chimera, resulting in deletions of CYP21A2, encoding 21-hydroxylase necessary for cortisol biosynthesis, and TNXB, encoding the extracellular matrix glycoprotein tenascin-X (TNX). This TNXA/TNXB chimera is characterized by a 120-bp deletion in exon 35 and results in TNXB haploinsufficiency, disrupted TGF-β signaling, and an Ehlers Danlos syndrome phenotype. The objective of the study was to determine the genetic status of TNXB and resulting protein defects in CAH patients with a CAH-X phenotype but not the previously described TNXA/TNXB chimera. Design, Settings, Participants, and Intervention: A total of 246 unrelated CAH patients were screened for TNXB defects. Genetic defects were investigated by Southern blotting, multiplex ligation-dependent probe amplification, Sanger, and next-generation sequencing. Dermal fibroblasts and tissue were used for immunoblotting, immunohistochemical, and coimmunoprecipitation experiments. The genetic and protein status of tenascin-X in phenotypic CAH-X patients was measured. Seven families harbor a novel TNXB missense variant c.12174C>G (p.C4058W) and a clinical phenotype consistent with hypermobility-type Ehlers Danlos syndrome. Fourteen CAH probands carry previously described TNXA/TNXB chimeras, and seven unrelated patients carry the novel TNXB variant, resulting in a CAH-X prevalence of 8.5%. This highly conserved pseudogene-derived variant in the TNX fibrinogen-like domain is predicted to be deleterious and disulfide bonded, results in reduced dermal elastin and fibrillin-1 staining and altered TGF-β1 binding, and represents a novel TNXA/TNXB chimera. Tenascin-X protein expression was normal in dermal fibroblasts, suggesting a dominant-negative effect. CAH-X syndrome is commonly found in CAH due to 21-hydroxylase deficiency and may result from various etiological mechanisms.

  4. Male genitoplasty for 46 XX congenital adrenal hyperplasia patients presenting late and reared as males

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    Shilpa Sharma

    2012-01-01

    Full Text Available Aim: To evaluate the clinical profile and management of 46 XX Congenital Adrenal Hyperplasia (CAH patients presenting with severe virilization and assigned a male gender. Materials and Methods: Of 173 children diagnosed with CAH at the Pediatric Intersex Clinic since 1980, seven children with CAH presented late with severe virilization and were reared as males. All of them were assigned the male sex with removal of the female adnexa. Six were treated with male genitoplasty. Appropriate hormonal supplementation was offered after puberty. Results: The mean age at presentation was 14.2 years (7 - 21. Six patients had presented after puberty, only one at seven years of age. Staged male genitoplasty comprising of chordee correction, male urethroplasty, and bilateral testicular prosthesis was performed. The female adnexa (uterus, ovaries, most of the upper vagina, and the fallopian tubes were removed. The mental makeup was masculine in six and bigender in one. Bilateral mastectomy was performed at puberty in all. Hormonal treatment comprised of glucocorticoids and testosterone. Six patients were comfortable with the outcome of the masculinizing genitoplasty. One had a short-sized phallus. One had repeated attacks of urinary tract infection arising from the retained lower vaginal pouch. Social adjustments were good in all, except in one who had a bigender mental makeup. Conclusion: CAH patients with severe virilization presenting late and reared as males are extremely rare. However, the assigned gender can be retained adequately as males, meeting the socioeconomic compulsions of the society. The results are satisfactory following appropriate surgical procedures and hormonal supplementation.

  5. Bone mineral density in young adult women with congenital adrenal hyperplasia

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    Nishant Raizada

    2016-01-01

    Full Text Available Background: There is equipoise regarding the status of bone mineral density (BMD in patients with congenital adrenal hyperplasia (CAH, where patients need to be on long-term low-dose steroids. Objective: We aimed to evaluate BMD at the hip, spine and forearm in women with CAH and compare it to healthy young adult women of the same age range. Subjects and Methods: Fifteen adult women with CAH with age ranging from 18 to 40 years (mean ± standard deviation = 27.5 ± 6.2 years underwent dual-energy X-ray absorptiometry along with laboratory evaluation. BMD at lumbar spine, hip, forearm along with T-scores were measured. Serum total calcium, phosphate, alkaline phosphatase, 25 hydroxy Vitamin D, intact parathyroid hormone, total testosterone, and dehydroepiandrosterone were assayed. History of any fractures in the past was taken. Fifteen healthy women in the same age range were taken as controls for comparison. Results: The BMD at hip (0.85 ± 0.02 g/cm2 in CAH was significantly lower as compared with controls (0.92 ± 0.03 g/cm2, P = 0.029. BMD at lumbar spine was also reduced (0.96 ± 0.02 vs. 1.03 ± 0.03, P = 0.057. The BMD at forearm was not significantly different between CAH and controls. The mean Vitamin D was 9.8 ng/ml (deficient range. There was no history of fractures in CAH. Conclusion: Young adult CAH women had lower BMD at spine and hip than healthy young adult women of the same age range. The forearm BMD was not different from controls. No change in fracture frequency was present. Patients with CAH being treated with steroids are at increased risk of osteopenia, and their bone health needs to be monitored.

  6. Gender of rearing and psychosocial aspect in 46 XX congenital adrenal hyperplasia

    Science.gov (United States)

    Gangaher, Arushi; Jyotsna, Viveka P.; Chauhan, Vasundhera; John, Jomimol; Mehta, Manju

    2016-01-01

    Background: In congenital adrenal hyperplasia (CAH) with ambiguous genitalia, assigning gender of rearing can be complex, especially If genitalia is highly virilized. Apart from karyotype, prenatal androgen exposure, patient's gender orientation, sociocultural, and parental influences play a role. The aim of this study was to assess gender dysphoria and psychosocial issues in patients of CAH raised as males and females. Materials and Methods: This is a cross-sectional study that includes patients (old and new) with CAH who were treated by us in the last 6 months. A semi-structured interview proforma was used to elicit history and psychosocial background of the patients. The clinical and biochemical details were noted. For psychological analysis, patients were screened for gender dysphoria using Parent Report Gender Identity Questionnaire for children Gender Identity/Gender Dysphoria Questionnaire for Adolescents and Adults. Results: We analyzed 22 46 XX CAH patients among which, 3 were reared as males and 19 as females. Among the 19 patients reared as females, 17 patients showed no gender dysphoria. Two patients revealed gender dysphoria as indicated by their marginally low scores on the gender dysphoria assessment. However, in view of current literature and the age groups of the patients, behavior of the 6-year-old patient can be best understood as being tomboyish. Gender dysphoria in the 22-year-old can be explained by the dominance of psychosocial factors and not hormones alone. Among the three patients reared as males, two prepubertal were satisfied with their male gender identity. The third patient, aged 32 years, had gender dysphoria when reared as a male that resolved when gender was reassigned as female and feminizing surgery was done. Conclusion: Gender assignment in 46 XX CAH is guided by factors such as degree of virilization of genitalia, gender orientation, patient involvement, sociocultural, and parental influences. PMID:27867895

  7. Gender of rearing and psychosocial aspect in 46 XX congenital adrenal hyperplasia

    Directory of Open Access Journals (Sweden)

    Arushi Gangaher

    2016-01-01

    Full Text Available Background: In congenital adrenal hyperplasia (CAH with ambiguous genitalia, assigning gender of rearing can be complex, especially If genitalia is highly virilized. Apart from karyotype, prenatal androgen exposure, patient's gender orientation, sociocultural, and parental influences play a role. The aim of this study was to assess gender dysphoria and psychosocial issues in patients of CAH raised as males and females. Materials and Methods: This is a cross-sectional study that includes patients (old and new with CAH who were treated by us in the last 6 months. A semi-structured interview proforma was used to elicit history and psychosocial background of the patients. The clinical and biochemical details were noted. For psychological analysis, patients were screened for gender dysphoria using Parent Report Gender Identity Questionnaire for children <12 years and Gender Identity/Gender Dysphoria Questionnaire for Adolescents and Adults. Results: We analyzed 22 46 XX CAH patients among which, 3 were reared as males and 19 as females. Among the 19 patients reared as females, 17 patients showed no gender dysphoria. Two patients revealed gender dysphoria as indicated by their marginally low scores on the gender dysphoria assessment. However, in view of current literature and the age groups of the patients, behavior of the 6-year-old patient can be best understood as being tomboyish. Gender dysphoria in the 22-year-old can be explained by the dominance of psychosocial factors and not hormones alone. Among the three patients reared as males, two prepubertal were satisfied with their male gender identity. The third patient, aged 32 years, had gender dysphoria when reared as a male that resolved when gender was reassigned as female and feminizing surgery was done. Conclusion: Gender assignment in 46 XX CAH is guided by factors such as degree of virilization of genitalia, gender orientation, patient involvement, sociocultural, and parental influences.

  8. Gender of rearing and psychosocial aspect in 46 XX congenital adrenal hyperplasia.

    Science.gov (United States)

    Gangaher, Arushi; Jyotsna, Viveka P; Chauhan, Vasundhera; John, Jomimol; Mehta, Manju

    2016-01-01

    In congenital adrenal hyperplasia (CAH) with ambiguous genitalia, assigning gender of rearing can be complex, especially If genitalia is highly virilized. Apart from karyotype, prenatal androgen exposure, patient's gender orientation, sociocultural, and parental influences play a role. The aim of this study was to assess gender dysphoria and psychosocial issues in patients of CAH raised as males and females. This is a cross-sectional study that includes patients (old and new) with CAH who were treated by us in the last 6 months. A semi-structured interview proforma was used to elicit history and psychosocial background of the patients. The clinical and biochemical details were noted. For psychological analysis, patients were screened for gender dysphoria using Parent Report Gender Identity Questionnaire for children Gender Identity/Gender Dysphoria Questionnaire for Adolescents and Adults. We analyzed 22 46 XX CAH patients among which, 3 were reared as males and 19 as females. Among the 19 patients reared as females, 17 patients showed no gender dysphoria. Two patients revealed gender dysphoria as indicated by their marginally low scores on the gender dysphoria assessment. However, in view of current literature and the age groups of the patients, behavior of the 6-year-old patient can be best understood as being tomboyish. Gender dysphoria in the 22-year-old can be explained by the dominance of psychosocial factors and not hormones alone. Among the three patients reared as males, two prepubertal were satisfied with their male gender identity. The third patient, aged 32 years, had gender dysphoria when reared as a male that resolved when gender was reassigned as female and feminizing surgery was done. Gender assignment in 46 XX CAH is guided by factors such as degree of virilization of genitalia, gender orientation, patient involvement, sociocultural, and parental influences.

  9. Ethnic disparity in 21-hydroxylase gene mutations identified in Pakistani congenital adrenal hyperplasia patients

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    Jabbar Abdul

    2011-02-01

    Full Text Available Abstract Background Congenital adrenal hyperplasia (CAH is a group of autosomal recessive disorders caused by defects in the steroid 21 hydroxylase gene (CYP21A2. We studied the spectrum of mutations in CYP21A2 gene in a multi-ethnic population in Pakistan to explore the genetics of CAH. Methods A cross sectional study was conducted for the identification of mutations CYP21A2 and their phenotypic associations in CAH using ARMS-PCR assay. Results Overall, 29 patients were analyzed for nine different mutations. The group consisted of two major forms of CAH including 17 salt wasters and 12 simple virilizers. There were 14 phenotypic males and 15 females representing all the major ethnic groups of Pakistan. Parental consanguinity was reported in 65% cases and was equally distributed in the major ethnic groups. Among 58 chromosomes analyzed, mutations were identified in 45 (78.6% chromosomes. The most frequent mutation was I2 splice (27% followed by Ile173Asn (26%, Arg 357 Trp (19%, Gln319stop, 16% and Leu308InsT (12%, whereas Val282Leu was not observed in this study. Homozygosity was seen in 44% and heterozygosity in 34% cases. I2 splice mutation was found to be associated with SW in the homozygous. The Ile173Asn mutation was identified in both SW and SV forms. Moreover, Arg357Trp manifested SW in compound heterozygous state. Conclusion Our study showed that CAH exists in our population with ethnic difference in the prevalence of mutations examined.

  10. [Recommendations for the diagnosis and treatment of classic forms of 21-hydroxylase-deficient congenital adrenal hyperplasia].

    Science.gov (United States)

    Rodríguez, Amparo; Ezquieta, Begoña; Labarta, José Igancio; Clemente, María; Espino, Rafael; Rodriguez, Amaia; Escribano, Aranzazu

    2017-08-01

    Congenital adrenal hyperplasia due to 21-hydroxylase deficiency is an autosomal recessive disorder caused by mutations in the CYP21A2 gene. Cortisol and aldosterone synthesis are impaired in the classic forms (adrenal insufficiency and salt-wasting crisis). Females affected are virilised at birth, and are at risk for genital ambiguity. In this article we give recommendations for an early as possible diagnosis and an appropriate and individualised treatment. A patient and family genetic study is essential for the diagnosis of the patient, and allows genetic counselling, as well as a prenatal diagnosis and treatment for future pregnancy. Copyright © 2016 Asociación Española de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

  11. Severe Short Stature in an Adolescent Male with Prader-Willi Syndrome and Congenital Adrenal Hyperplasia: A Therapeutic Conundrum

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    Meredith Wasserman

    2017-01-01

    Full Text Available Congenital adrenal hyperplasia (CAH due to 21-hydroxylase deficiency results in excess androgen production which can lead to early epiphyseal fusion and short stature. Prader-Willi syndrome (PWS is a genetic disorder resulting from a defect on chromosome 15 due to paternal deletion, maternal uniparental disomy, or imprinting defect. Ninety percent of patients with PWS have short stature. In this article we report a patient with simple-virilizing CAH and PWS who was overtreated with glucocorticoids for CAH and not supplemented with growth hormone for PWS, resulting in a significantly short adult height.

  12. Delayed diagnosis of congenital adrenal hyperplasia with salt wasting due to type II 3beta-hydroxysteroid dehydrogenase deficiency

    DEFF Research Database (Denmark)

    Johannsen, Trine H; Mallet, Delphine; Dige-Petersen, Harriet

    2005-01-01

    to the diagnosis) and elevated Delta5-/Delta4-steroid ratios. Sequencing of the type II 3beta-HSD gene documented that both girls were compound heterozygotes for T181I and 1105delA mutations. Retrospectively, elevated levels of 17alpha-hydroxyprogesterone were found on blood spots from Guthrie's test....... There is no previous report of the combination of SW and premature pubarche due to mutations in the type II 3beta-HSD gene. Because neonatal diagnosis could have prevented life-threatening crises in these girls, this report further supports the benefits for neonatal screening for congenital adrenal hyperplasia...

  13. Should we question early feminizing genitoplasty for patients with congenital adrenal hyperplasia and XX karyotype?

    Science.gov (United States)

    Binet, A; Lardy, H; Geslin, D; Francois-Fiquet, C; Poli-Merol, M L

    2016-03-01

    There is a wide difference of opinion between the medical-surgical community and advocacy group regarding Disorders of Sexual Development (DSD) secondary to congenital adrenal hyperplasia (CAH) being ranked in the intersex category. This rupture is even more evident when the issue of genitoplasty is brought up. For physicians it is obvious and unequivocal that a person with CAH and an XX karyotype has a female gender identity, whereas associations tend to rank persons with CAH in the intersex category and advocate holding-off on surgical management. A retrospective case study vs. control group, spanning over 40years, included 21 patients who were treated in 3 different centers. Each patient and their parents were contacted independently and interviewed regarding interpersonal relationships, psychological impact of genitoplasty, gender identity and opinion on optimal care management for this disorder. Three couples controls (parent-child) per CAH patients were used and matched according to age, sex assigned at birth and ethnic origin. Sex assigned at birth seemed to concord with the gender identity perceived by the patients in 85.7% of cases. In fact, 89.7% of patients and 100% of parents felt that feminizing genitoplasty should be performed within the first year of life. There is however a significant difference compared to controls who felt that surgical management should occur later on in life. No difference was highlighted during childhood regarding parents-child relationships or social integration. However, during adolescence, the parents-child relationship tended to be significantly more painful for the CAH group. Integrating their parenting role was significantly harder for patients in the CAH-DSD group. In the population of CAH-DSD patients who had genitoplasty the level of sexual fulfillment was not lower to the one reported by the control group. Female sex assignment seems legitimate according to this study and the development of gender identity in these

  14. Congenital Adrenal Hyperplasia in an Elite Female Soccer Player; What Sports Medicine Clinicians Should Know about This?

    Science.gov (United States)

    Angoorani, Hooman; Haratian, Zohreh; Halabchi, Farzin

    2012-09-01

    Congenital Adrenal Hyperplasia (CAH) refers to a group of congenital conditions characterized by disordered cortisol synthesis. The correlation between CAH and sports performance has been less studied before and there is very limited information regarding the impacts of this congenital disease on sports performance. Probably, there are some limitations for patients who suffer from CAH in sports, but at the same time, they may enjoy some advantage due to the probable effect of endogenous hyperandrogenism on their exercise performance. The case is a 14 - year old girl with male phenotype who is a known case of congenital adrenal hyperplasia. She plays in the women's national soccer team of under 16. She has been in the first division league of indoor soccer for 4 years and was also selected in the preparation training camp of women's football team for Singapore's youth Olympic Games. Her illness and dependence on corticosteroid have caused some concerns for her participation in the international competitions of women. However, following consultations with the Therapeutic Use Exemption (TUE) Committee of games organization, she received TUE to use corticosteroid only within the games period. Despite all her problems, she is now playing in the Second Division League of indoor soccer. A female adolescent with CAH may compete at the high level of outdoor and indoor soccer. However, there are many questions regarding the advantages and disadvantages of this congenital disorder and its treatment on sports related issues.

  15. A Case of Bilateral Testicular Tumors Subsequently Diagnosed as Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency

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    Yan-Kun Sha

    2016-12-01

    Full Text Available 21-hydroxylase deficiency (21-OHD caused congenital adrenal hyperplasia (CAH is a group of autosomal recessive genetic disorders resulting from mutations in genes involved with cortisol (CO synthesis in the adrenal glands. Testicular adrenal rest tumors (TARTs are rarely the presenting symptoms of CAH. Here, we describe a case of simple virilizing CAH with TARTs, in a 15-year-old boy. The patient showed physical signs of precocious puberty. The levels of blood adrenocorticotropic hormone (ACTH, urinary 17-ketone steroids (17-KS, dehydroepiandrosterone sulfate (DHEA-S, and serum progesterone (PRGE were elevated, whereas those of follicle-stimulating hormone (FSH, luteinizing hormone (LH, and CO were reduced. Computed tomography (CT of the adrenal glands and magnetic resonance imaging (MRI of the testes showed a soft tissue density (more pronounced on the right side and an irregularly swollen mass (more pronounced on the left side, respectively. Pathological examination of a specimen of the mass indicated polygonal/circular eosinophilic cytoplasm, cord-like arrangement of interstitial cells, and lipid pigment in the cytoplasm. Immunohistochemistry results precluded a diagnosis of Leydig cell tumors. DNA sequencing revealed a hackneyed homozygous mutation, I2g, on intron 2 of the CYP21A2 gene. The patient’s symptoms improved after a three-month of dexamethasone therapy. Recent radiographic data showed reduced hyperplastic adrenal nodules and testicular tumors. A diagnosis of TART should be considered and prioritized in CAH patients with testicular tumors. Replacement therapy using a sufficient amount of dexamethasone in this case helps combat TART.

  16. The long term outcome of feminizing genital surgery for congenital adrenal hyperplasia: anatomical, functional and cosmetic outcomes, psychosexual development, and satisfaction in adult female patients.

    NARCIS (Netherlands)

    Stikkelbroeck, M.M.L.; Beerendonk, C.C.M.; Willemsen, W.N.P.; Schreuders-Bais, C.; Feitz, W.F.J.; Rieu, P.N.M.A.; Hermus, A.R.M.M.; Otten, B.J.

    2003-01-01

    BACKGROUND: There are only a few reports analyzing the long term outcome of feminizing surgery in females with congenital adrenal hyperplasia (CAH). Such analysis is crucial to evaluate the treatment and to make necessary adjustments. STUDY OBJECTIVES: To evaluate the adult outcome after feminizing

  17. Physical, social and societal functioning of children with congenital adrenal hyperplasia (CAH and their parents, in a Dutch population

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    Sanches Sarita A

    2012-02-01

    Full Text Available Abstract Background Most research concerning congenital adrenal hyperplasia (CAH and related conditions caused by primary adrenal insufficiency, such as Addison's or Cushing's disease, has focused on medical aspects rather than on patients' quality of life. Therefore, our objective was to investigate the physical, social and societal functioning of children with CAH and their parents in a Dutch population. Methods The study is descriptive and cross-sectional. Self-designed questionnaires, based on questionnaires developed in the Netherlands for different patient groups, were sent to parents of children with CAH between 0 and 18 years old. Participants were recruited through the Dutch patient group for Adrenal Disease (NVACP and six hospitals in the Netherlands. Three different questionnaires were designed for parents: for children aged 0 - 4, aged 4 - 12 and aged 12 - 18. Additionally, a fourth questionnaire was sent to adolescents with CAH aged 12 - 18. Main outcome measures were experienced burden of the condition, self-management and participation in several areas, such as school and leisure time. Results A total of 106 parents returned the questionnaire, 12 regarding pre-school children (0-4 years, 63 regarding primary school children (4-12 years, and 32 regarding secondary school children (12-18 years, combined response rate 69.7%. Also, 24 adolescents returned the questionnaire. Children and adolescents with CAH appear to be capable of self-management at a young age. Experienced burden of the condition is low, although children experience several health related problems on a daily basis. Children participate well in school and leisure time. Few children carry a crisis card or emergency injection with them. Conclusions Overall, our research shows that, according to their parents, children with CAH experience few negative effects of the condition and that they participate well in several areas such as school and leisure time. However

  18. Stigma in Medical Settings As Reported Retrospectively by Women With Congenital Adrenal Hyperplasia (CAH) for Their Childhood and Adolescence.

    Science.gov (United States)

    Meyer-Bahlburg, Heino F L; Khuri, Jananne; Reyes-Portillo, Jazmin; New, Maria I

    2017-06-01

    To perform a qualitative study of stigma experienced in medical settings by children and adolescents with congenital genital ambiguity (CGA). 62 women with classical congenital adrenal hyperplasia (CAH) of variable severity took part in a qualitative retrospective interview that focused on the impact of CAH and its medical treatment, with an emphasis on childhood and adolescence. Categorization of stigmatization was based on deductive content analysis of the interview transcripts. Many women recalled experiencing the genital examinations in childhood and adolescence as adverse, stigmatizing events, leading to avoidance reactions and self-perception as abnormal, particularly when the examinations included groups of trainees. Some women also experienced as adverse the nonverbal and verbal reactions of individual physicians who were unfamiliar with CGA. Genital examinations constitute salient events for children and adolescents with CGA. They are easily experienced as strongly stigmatizing, especially when combined with teaching.

  19. Genetics Home Reference: congenital adrenal hyperplasia due to 11-beta-hydroxylase deficiency

    Science.gov (United States)

    ... of the kidneys and produce a variety of hormones that regulate many essential functions in the body. In people with CAH due to 11-beta-hydroxylase deficiency, the adrenal glands produce excess androgens, which are male sex hormones. There are two types of CAH ...

  20. Familial glucocorticoid receptor haploinsufficiency by non-sense mediated mRNA decay, adrenal hyperplasia and apparent mineralocorticoid excess.

    Directory of Open Access Journals (Sweden)

    Jérôme Bouligand

    Full Text Available Primary glucocorticoid resistance (OMIM 138040 is a rare hereditary disease that causes a generalized partial insensitivity to glucocorticoid action, due to genetic alterations of the glucocorticoid receptor (GR. Investigation of adrenal incidentalomas led to the discovery of a family (eight affected individuals spanning three generations, prone to cortisol resistance, bilateral adrenal hyperplasia, arterial hypertension and hypokalemia. This phenotype exacerbated over time, cosegregates with the first heterozygous nonsense mutation p.R469[R,X] reported to date for the GR, replacing an arginine (CGA by a stop (TGA at amino-acid 469 in the second zinc finger of the DNA-binding domain of the receptor. In vitro, this mutation leads to a truncated 50-kDa GR lacking hormone and DNA binding capacity, devoid of hormone-dependent nuclear translocation and transactivation properties. In the proband's fibroblasts, we provided evidence for the lack of expression of the defective allele in vivo. The absence of detectable mutated GR mRNA was accompanied by a 50% reduction in wild type GR transcript and protein. This reduced GR expression leads to a significantly below-normal induction of glucocorticoid-induced target genes, FKBP5 in fibroblasts. We demonstrated that the molecular mechanisms of glucocorticoid signaling dysfunction involved GR haploinsufficiency due to the selective degradation of the mutated GR transcript through a nonsense-mediated mRNA Decay that was experimentally validated on emetine-treated propositus' fibroblasts. GR haploinsufficiency leads to hypertension due to illicit occupation of renal mineralocorticoid receptor by elevated cortisol rather than to increased mineralocorticoid production reported in primary glucocorticoid resistance. Indeed, apparent mineralocorticoid excess was demonstrated by a decrease in urinary tetrahydrocortisone-tetrahydrocortisol ratio in affected patients, revealing reduced glucocorticoid degradation by

  1. Congenital lipoid adrenal hyperplasia caused by a novel splicing mutation in the gene for the steroidogenic acute regulatory protein.

    Science.gov (United States)

    González, Alexis A; Reyes, M Loreto; Carvajal, Cristian A; Tobar, Jaime A; Mosso, Lorena M; Baquedano, Paulina; Solar, Antonieta; Venegas, Alejandro; Fardella, Carlos E

    2004-02-01

    Steroidogenic acute regulatory protein (StAR) plays a crucial role in the transport of cholesterol from the cytoplasm to the inner mitochondrial membrane, facilitating its conversion to pregnenolone by cytochrome P450scc. Its essential role in steroidogenesis was demonstrated after observing that StAR gene mutations gave rise to a potentially lethal disease named congenital lipoid adrenal hyperplasia, in which virtually no steroids are produced. We report here a 2-month-old female patient, karyotype 46XY, who presented with growth failure, convulsions, dehydration, hypoglycemia, hyponatremia, hypotension, and severe hyperpigmentation suggestive of adrenal insufficiency. Serum cortisol, 17OH-progesterone, dehydroepiandrosterone sulfate, testosterone, 17OH-pregnenolone, and aldosterone levels were undetectable in the presence of high ACTH and plasma renin activity levels. Immunohistochemical analysis of testis tissues revealed the absence of StAR protein. Molecular analysis of StAR gene demonstrated a homozygous G to T mutation within the splice donor site of exon 1 (IVS1 + 1G>T). Her parents and one brother were heterozygous for this mutation. In vitro analysis of the mutation was performed in COS cells transfected with minigenes coding regions spanning exon-intron 1 to 3 carrying the mutant and the wild-type sequences. RT-PCR analyses of the mutant gene showed an abnormal mRNA transcript of 2430 bp (normal size 433 bp). Sequence analysis of the mutant mRNA demonstrated the retention of intron 1. Immunolocalization of the StAR minigene product detected the peptide in the mitochondria of COS cells transfected with the wild-type minigene but not in those transfected with the mutant minigene. We conclude that this mutation gives rise to a truncated StAR protein, which lacks an important N-terminal region and the entire lipid transfer domain.

  2. Clinical characteristics of Taiwanese children with congenital adrenal hyperplasia due to 21-hydroxylase deficiency detected by neonatal screening.

    Science.gov (United States)

    Liu, Shih-Yao; Lee, Cheng-Ting; Tung, Yi-Ching; Chien, Yin-Hsiu; Hwu, Wuh-Liang; Tsai, Wen-Yu

    2018-02-01

    Neonatal screening for congenital adrenal hyperplasia (CAH) has been conducted in Taiwan since 2000. This study aimed to determine the clinical characteristics of Taiwanese children with CAH due to 21-hydroxylase deficiency (21-OHD) detected by neonatal screening. From 2000 to 2015, 26 neonates (14 boys and 12 girls) with classic 21-OHD detected by neonatal screening and confirmed at National Taiwan University Hospital were enrolled. Among them, 22 were diagnosed as salt wasting (SW) type and four as simple virilizing (SV) type. Through a review of medical records, their clinical presentations, laboratory data, and molecular studies were analyzed. The most common manifestation was hyperpigmentation. All female neonates regardless of 21-OHD type had atypical genitalia, clitoromegaly, and posterior labial fusion. All of the patients had baseline serum 17-hydroxyprogesterone levels higher than normal. Of the 26 patients, 24 had elevated adrenocorticotropic hormone levels, but only four had low serum cortisol levels. The median baseline adrenocorticotropic hormone, 17-hydroxyprogesterone, and androstenedione levels were significantly higher in patients with SW than in those with SV 21-OHD. All patients with SW 21-OHD had elevated plasma renin activity. The most frequent SW 21-OHD mutations were c.293-13C>G and gene deletion, whereas Ile173Asn and c.293-13C>G were the most frequently detected in SV 21-OHD. In Taiwan, neonatal screening effectively leads to the early diagnosis of CAH and reduces fatal adrenal crisis in neonates. This study may provide physicians with a better understanding of the clinical findings among children with early-diagnosed CAH, allowing for better care in the future. Copyright © 2017. Published by Elsevier B.V.

  3. [Congenital adrenal hyperplasia: a qualitative study on sex definition and redesignation dilation surgery and psychological support (part II)].

    Science.gov (United States)

    Telles-Silveira, Mariana; Tonetto-Fernandes, Vânia F; Schiller, Paulo; Kater, Claudio E

    2009-12-01

    To identify relevant questions related to sex definition and re-designation and reconstructive surgery in patients with congenital adrenal hyperplasia (CAH), and to understand the role of the psychologist in providing care for these patients. We selected 21 subjects: 7 pediatric endocrinologists from 5 Brazilian Public Health System institutions, 9 parents and 6 patients with CAH, according to a qualitative research model. In this paper, 3 of the studied categories are analyzed: 'sex definition and re-designation', 'reconstructive surgery/vaginal dilation', and 'psychology'. Parents' main anguish relates to the situation of an unnamed sex at birth, whereas sex re-designation was distressful to physicians. A sense of loneliness when dealing with the disease and treatment was a common anguish among patients; dilation procedures were the major complaint. In general, physicians recommend that genital reconstructive surgery be performed early on to avoid future trauma. In such a complex scenario, it is remarkable that not all the reference service staff have a psychologist on duty. Difficulties to deal with questions involving sexuality were evident and dilation procedures are an additional source of trauma for these patients.

  4. Laparoscopic-assisted vaginal pull-through: A new approach for congenital adrenal hyperplasia patients with high urogenital sinus

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    Jacques Birraux

    2015-01-01

    Full Text Available Background: To open vaginal cavity to the pelvic floor is part of surgical treatment for urogenital sinus (UGS in girls with congenital adrenal hyperplasia (CAH. For high UGS, this operative procedure can be challenging and may jeopardise urinary continence. Combined perineal and laparoscopic approaches could be useful to minimise perineal dissection and to facilitate the vaginal lowering. Patients and Methods: We report the procedure of a laparoscopic-assisted vaginal pull-through for supra-sphincteric UGS in a 5-year-old girl with CAH. Laparoscopic dissection of the vagina from the posterior wall of the bladder and urethra, division of the confluence and vaginal pull-through to the perineum are described. Discussion: The technique is derived from laparoscopic-assisted treatment for high ano-rectal malformations. Compared with current procedures for treatment for high UGS, laparoscopic-assisted approach allows mobilising vagina with minimal dissection of perineum and complete preservation of urethra. Another major advantage is to provide a direct vision for dissection of the space between rectum and urethra prior to vaginal pull-through. Conclusion: Laparoscopic-assisted vaginal pull-through appears to be an interesting approach for high UGS in CAH patients, reducing dissection and risk of urinary incontinence. This new approach needs to be strengthened by other cases.

  5. Male gender identity in children with 46,XX DSD with congenital adrenal hyperplasia after delayed presentation in mid-childhood.

    Science.gov (United States)

    Chowdhury, Tanvir Kabir; Laila, Kamrun; Hutson, John M; Banu, Tahmina

    2015-12-01

    Girls with congenital adrenal hyperplasia (CAH) diagnosed at birth have some masculine behaviors but rarely convert to male gender. In developing countries, however, diagnosis and treatment (with secondary androgen suppression) are delayed. We aimed to assess effect of delayed treatment of CAH on gender identity. As part of a cross-sectional, case-control study of children with disorders of sex development (DSD), there were 11 patients with CAH. Patients and caregivers answered a questionnaire about gender identity, and behavior was assessed by observing toy play. Patients were examined for Prader score and gender identity. Of 11 CAH patients initially raised as girls, 3 (27%) had converted to male gender at presentation (5, 9, 9years) (Prader 3, 4, 4). Of the remaining 8 patients, one 4-year-old (Prader 2) had a male gender identity score. The remaining girls (2-13years, mean 8.1) (Prader 1-3) had gender identity scores in the female range. One third (4/11) of CAH patients presenting in mid-childhood had male gender identity scores, and ¾ had assumed male gender role. Although social and cultural factors are important in developing countries, this result suggests that delayed treatment may trigger male gender identity, and delayed female genital surgery may be unwise. Copyright © 2015 Elsevier Inc. All rights reserved.

  6. Microfilter paper method for 17α-hydroxyprogesterone radioimmunoassay: its application for rapid screening for congenital adrenal hyperplasia

    International Nuclear Information System (INIS)

    Pang, S.; Hotchkiss, J.; Drash, A.L.; Levine, L.S.; New, M.I.

    1977-01-01

    A new micromethod for measuring a steroid in blood collected on filter paper has been developed. The method is easy and rapid and has the specificity, accuracy and precision of RIA in whole plasma. Less than 20 μl of blood is required, and, therefore, samples may be obtained with heel prick. This method has been applied to the determination of 17α-hydroxyprogesterone (17α-OH-P) for screening patients with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. There was excellent correlation (r = .94) between the values of 17α-OH-P obtained by microfilter paper method and those from plasma samples of cord (40 +- 13 ng/ml) and neonatal blood (<3.6 ng/ml) in normal infants. In six neonates at risk for CAH the diagnosis was made utilizing the microfilter paper method. 17α-OH-P concentrations were highly elevated in both filter paper eluates of whole blood (67 to 360 ng/ml of plasma) and simultaneously obtained plasma concentration (74 to 395 ng/ml) in affected infants. The concentrations of 17α-OH-P remained unchanged in dried filter paper blood when stored at room temperature for up to 21 days. Thus, filter paper with dried blood may be sent for steroid assay by mail. The ease with which samples may be transported and the minute amount of sample necessary make this method a promising screening test for CAH

  7. Sexual orientation and medical history among Iranian people with Complete Androgen Insensitivity Syndrome and Congenital Adrenal Hyperplasia.

    Science.gov (United States)

    Khorashad, Behzad S; Roshan, Ghasem M; Reid, Alistair G; Aghili, Zahra; Hiradfar, Mehran; Afkhamizadeh, Mozhgan; Talaei, Ali; Aarabi, Azadeh; Ghaemi, Nosrat; Taghehchian, Negin; Saberi, Hedieh; Farahi, Nazanin; Abbaszadegan, Mohammad Reza

    2017-01-01

    To report sexual orientation, relationship status and medical history of Iranian people with Differences of Sex Development (DSD) who were raised female. Our participants consisted of nineteen 46,XY individuals with Complete Androgen Insensitivity Syndrome (CAIS) and eighteen 46,XX individuals with Congenital Adrenal Hyperplasia (CAH) who were raised as females and older than 13years. As well as their relationship status and detailed medical history, an expert psychiatrist assessed their sexual orientation by a semi-structured psychiatric interview with them and, where applicable, their parents. Five percent of CAH participants and 42% of CAIS participants were in a relationship, which was significantly different. All CAH individuals had been diagnosed at birth; 89% of CAIS had been diagnosed after puberty and due to primary amenorrhea and 11% were diagnosed in childhood due to inguinal hernia. Genital reconstructive surgery had been performed in 100% of CAH participants and 37% of CAIS. Regarding sexual contact experiences and sexual fantasies (androphilic, gynephilic or both), no significant differences were found. However, CAH females had significantly more gynephilic dreams (P=0.045). This study, notable as one of the rare from a non-western culture, described sexual, medical and socioeconomic status of 46,XX CAH and 46,XY CAIS individuals living in Iran. Although broadly in line with previous findings from Western cultures, Iranian CAH individuals had fewer romantic relationships, but in contrast to previous studies their sexual orientation was only different from CAIS in the contents of sexual dreams. Copyright © 2016 Elsevier Inc. All rights reserved.

  8. Sexual difference in bone geometry of adult patients with classical congenital adrenal hyperplasia: data using peripheral quantitative computed tomography.

    Science.gov (United States)

    Bechtold, Susanne; Beyerlein, Andreas; Bonfig, Walter; Dalla Pozza, Robert; Putzker, Stephanie; Otto, Ragna; Schmidt, Heinrich; Schwarz, Hans Peter

    2014-01-01

    Glucocorticoid treatment may influence bone and muscle development in patients with congenital adrenal hyperplasia (CAH). This study evaluated bone mineral density (BMD), bone geometry and muscle mass. 73 adult patients with classical CAH were followed. BMD, bone geometry and muscle mass were measured using peripheral quantitative computed tomography (pQCT). Glucocorticoid-equivalent doses throughout life were calculated and at the time pQCT androgen levels were measured. In males the mean standard deviation (SD) score for trabecular BMD (-0.33 ± 0.71) was reduced, whereas mean cortical BMD (1.05 ± 1.11) was elevated. Mean total (0.86 ± 1.12) and medullary cross-sectional area (CSA; 1.12 ± 1.17) were significantly increased (p difference with enlarged total and medullary CSA in females, whereas in males trabecular BMD was reduced and cortical BMD elevated. Cortical thickness and muscle CSA were reduced in all CAH patients with a possible long-term impact on bone development and stability. Monitoring of bone and muscle development might be warranted. © 2014 S. Karger AG, Basel.

  9. Syndrome-Related Stigma in the General Social Environment as Reported by Women with Classical Congenital Adrenal Hyperplasia.

    Science.gov (United States)

    Meyer-Bahlburg, Heino F L; Reyes-Portillo, Jazmin A; Khuri, Jananne; Ehrhardt, Anke A; New, Maria I

    2017-02-01

    Stigma defined as "undesired differentness" (Goffman, 1963) and subtyped as "experienced" or "enacted," "anticipated," and "internalized" has been documented for patients with diverse chronic diseases. However, no systematic data exist on the association of stigma with somatic intersexuality. The current report concerns women with classical congenital adrenal hyperplasia (CAH), the most prevalent intersex syndrome, and provides descriptive data on CAH-related stigma as experienced in the general social environment (excluding medical settings and romantic/sexual partners) during childhood, adolescence, and adulthood. A total of 62 adult women with classical CAH [41 with the salt-wasting (SW) variant and 21 with the simple-virilizing (SV) variant] underwent a qualitative retrospective interview, which focused on the impact of CAH and its medical treatment on many aspects of women's lives. Deductive content analysis was performed on the transcribed texts. The women's accounts of CAH-related stigma were identified and excerpted as vignettes, and the vignettes categorized according to social context, stigma type, and the associated features of the CAH condition. Nearly two-thirds of women with either variant of CAH provided stigma vignettes. The vignettes included all three stigma types, and most involved some somatic or behavioral feature related to sex or gender. Stigma situations were reported for all ages and all social contexts of everyday life: family, peers, colleagues at work, strangers, and the media. We conclude that there is a need for systematic documentation of stigma in intersexuality as a basis for the development of improved approaches to prevention and intervention.

  10. Metabolic profile and cardiovascular risk factors in adult patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency

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    Mouna Feki Mnif

    2012-01-01

    Full Text Available Background: In congenital adrenal hyperplasia (CAH, long-term glucocorticoid treatment coupled with increased androgens may lead to undesirable metabolic effects. The aim of our report was to determine the prevalence of metabolic abnormalities and cardiovascular risk factors in a population of adult patients with CAH due to 21 hydroxylase deficiency. Materials and Methods: Twenty-six patients (11 males and 15 females, mean age ± SD=27.4±8.2 years were recruited. Anthropometry, body composition, metabolic parameters and cardiovascular risk factors were studied. Results: Obesity (overweight included was noted in 16 patients (61.5%, with android distribution in all cases. Bioelectrical impedance showed increased body fat mass in 12 patients (46.1%. Lipid profile alterations and carbohydrate metabolism disorders were detected in seven (26.9% and five (19.2% patients respectively. Moderate hepatic cytolysis, associated with hepatic steatosis, was found in one patient. Seven patients (27% had insulin resistance. Ambulatory blood pressure monitoring showed abnormalities in six patients (23%. Increased carotid intima media thickness was found in 14 patients (53.8%. Conclusion: Adult CAH patients tend to have altered metabolic parameters and a higher prevalence of cardiovascular risk factors. Lifelong follow-up, lifestyle modifications, and attempts to adjust and reduce the glucocorticoid doses seem important.

  11. Surgical Treatment After Failed Primary Correction of Urogenital Sinus in Female Patients with Virilizing Congenital Adrenal Hyperplasia.

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    Maria Helena Palma Sircili

    2016-10-01

    Full Text Available Purpose: Genital reconstruction in female patients with virilizing congenital adrenal hyperplasia (CAH is very challenging. Our aim was to evaluate the techniques employed to treat complications after failure of primary urogenital sinus (UGS surgery, as well as the result of these reoperations.Patients and Methods: Twenty girls with virilizing CAH who were previously submitted to genitoplasty in our service and elsewhere had recurrent UGS stenosis and vaginal introitus stenosis that required surgical treatment. The main symptoms were recurrent urinary tract infection (UTI in nine, dyspareunia in six and hematocolpos in three (two associated with sepsis. The anatomical findings were the persistence of UGS with stenosis in 17 patients and vaginal introitus stenosis in three. The mean age at procedure was 15.2 yrs., averaging 13.1 yrs. after the first surgery. The surgical techniques employed were: isolated perineal flap in 17 patients and perineal flap with partial mobilization of UGS in three. The mean follow-up after the procedure was 4.8 years (varying from 1 to 17yrs.Results: Vaginal dilations were performed after surgery in 15 patients. Good functional and anatomical results were obtained in 15 patients, with vaginal introitus amenable to dilators of 3.0 cm in diameter. Five patients with high vaginal insertion had recurrent vaginal stenosis and required a surgical revision. No patients presented menstrual obstruction or UTI after surgery. Eight of the 15 adult patients are sexually active. Conclusion: The reoperation to treat failed primary UGS treatment using Y-V flap and partial mobilization techniques associated to vaginal dilations, promoted good anatomical and functional results with low morbidity in 75% of the patients.□

  12. Comparison of different glucocorticoid regimens in the management of classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

    Science.gov (United States)

    Ajish, T P; Praveen, V P; Nisha, B; Kumar, Harish

    2014-11-01

    There are recommendations regarding the total dose of hydrocortisone to be administered in the treatment of classical congenital adrenal hyperplasia (CAH) to achieve the twin objectives of glucocorticoid replacement and control of hyperandrogenism. However, there is evidence gap regarding the breakup, timing and type of the steroid regimen. Efficacy of three different glucocorticoid regimens having the same total dose of steroid, differing in either the timing or type of evening steroid administered, in achieving biochemical control of the disease was assessed. The study was done in 13 prepubertal children with classical CAH over a 6-month period with 2 months devoted to each regimen. We used a prospective cross-over design using 10-15 mg/m(2) total dose of hydrocortisone. Two-fifths of the total dose of hydrocortisone was administered in the morning and one-fifth of the total dose was administered at noon in all the regimens. The regimens differed in the timing of the evening dose of hydrocortisone, 06.00-07.00 pm in regimen 1 and 09.00-10.00 pm in regimen 2. The third regimen had the evening dose of hydrocortisone replaced by an equivalent dose of prednisolone suspension which was administered at 10.00 pm. Serum 17-hydroxyprogesterone and testosterone levels were compared to assess the efficacy of treatment regimens. The three different regimens were found to be similar in their ability to control 17-hydroxyprogesterone and testosterone levels. The percentage of patients with predefined criteria for biochemically controlled disease was similar in all the three regimens. However, there was a trend toward better control of 17-hydroxyprogesterone levels in patients receiving evening dose of prednisolone. There is no significant advantage in administering the hydrocortisone dose late at night in patients with classical CAH.

  13. Gender role behavior, sexuality, and psychosocial adaptation in women with congenital adrenal hyperplasia due to CYP21A2 deficiency.

    Science.gov (United States)

    Frisén, Louise; Nordenström, Anna; Falhammar, Henrik; Filipsson, Helena; Holmdahl, Gundela; Janson, Per Olof; Thorén, Marja; Hagenfeldt, Kerstin; Möller, Anders; Nordenskjöld, Agneta

    2009-09-01

    Gender-atypical behavior has been described in young girls as well as in women with congenital adrenal hyperplasia (CAH) due to a CYP21A2 deficiency. The aim of the study was to assess health-related, psychosexual, and psychosocial parameters and correlate the results to CYP21A2 genotype. Sixty-two Swedish women with CAH and age-matched controls completed a 120-item questionnaire and a validated quality of life instrument [psychological general well-being (PGWB) formula] to identify psychosexual and psychosocial parameters. The patients were divided into four CYP21A2 genotype groups. The women with CAH held more male-dominant occupations (30%) compared to controls (13%) (P = 0.04), especially those in the null genotype group (55%) (P = 0.006). They also reported a greater interest in rough sports (74%) compared to controls (50%) (P = 0.007). Eight women with CAH (14%) reported a prime interest in motor vehicles, compared to none of the controls (P = 0.002). Non-heterosexual orientation was reported by 19% of women with CAH (P = 0.005), 50% in the null genotype group (P = 0.0001), 30% in I2 splice (NS), and 5% in I172N (NS). PGWB total score did not differ between patients and controls. We identified increased gender-atypical behavior in women with CAH that could be correlated to the CYP21A2 genotype. This speaks in favor of dose-dependent effects of prenatal androgens on the development of higher brain functions. The impact of the disease on upbringing and interpersonal relationships did not correlate with disease severity, indicating that other factors, such as coping strategies, are important for psychosocial adaptation. This illustrates the need for psychological support to parents and patients.

  14. Impact of food, alcohol and pH on modified-release hydrocortisone developed to treat congenital adrenal hyperplasia.

    Science.gov (United States)

    Karunasena, Nayananjani; Margetson, Daniel N; Neal, Greg; Whitaker, Martin J; Ross, Richard Jm

    2017-04-01

    We developed a modified-release hydrocortisone, Chronocort, to replace the cortisol rhythm in patients with congenital adrenal hyperplasia. Food, alcohol and pH affect drug absorption, and it is important to assess their impact when replicating a physiological rhythm. In vitro dissolution to study impact of alcohol and pH on Chronocort. A phase 1, three-period, cross over study in 18 volunteers to assess the impact of food on Chronocort and to compare bioavailability to immediate-release hydrocortisone. In vitro dissolution of Chronocort was not affected by gastrointestinal pH up to 6.0 nor by an alcohol content up to 20% v/v. Food delayed and reduced the rate of absorption of Chronocort as reflected by a longer T max (fed vs fasted: 6.75 h vs 4.5 h, P  = 0005) and lower C max (549.49 nmol/L vs 708.46 nmol/L, ratio 77% with CI 71-85). Cortisol exposure was similar in fed and fasted state: Geo LSmean ratio (CI) AUC 0t for fed/fasted was 108.33% (102.30-114.72%). Cortisol exposure was higher for Chronocort compared to immediate-release hydrocortisone: Geo LSmean ratios (CI) 118.83% (111.58-126.54%); however, derived free cortisol showed cortisol exposure CIs were within 80.0-125.0%: Geo LSmean ratio (CI) for AUC 0t 112.73% (105.33-120.65%). Gastric pH ≤6.0 and alcohol do not affect hydrocortisone release from Chronocort. Food delays Chronocort absorption, but cortisol exposure is similar in the fasted and fed state and exposure as assessed by free cortisol is similar between Chronocort and immediate-release hydrocortisone. © 2017 The authors.

  15. P450XXI (steroid 21-hydroxylase) gene deletions are not found in family studies of congenital adrenal hyperplasia

    International Nuclear Information System (INIS)

    Matteson, K.J.; Phillips, J.A. III; Miller, W.L.; Chung, B.C.; Orlando, P.J.; Frisch, H.; Ferrandez, A.; Burr, I.M.

    1987-01-01

    Congenital adrenal hyperplasia (CAH) is a common genetic disorder due to defective 21-hydroxylation of steroid hormones. The human P450XXIA2 gene encodes cytochrome P450c21 [steroid 21-monooxygenase (steroid 21-hydroxylase)], which mediates 21-hydroxylation. The P450XXIA2 gene may be distinguished from the duplicated P450XXIA1 pseudogene by cleavage with the restriction endonuclease Taq I, with the XXIA2 gene characterized by a 3.7-kilobase (kb) fragment and the XXIA1 pseudogene characterized by a 3.2-kb fragment. Restriction endonuclease mapping by several laboratories has suggested that deletion of the P450XXIA2 gene occurs in about 25% of patients with CAH, as their genomic DNA lacks detectable 3.7-kb Taq I fragments. The authors have cloned human P450c21 cDNA and used it to study genomic DNA prepared from 51 persons in 10 families, each of which includes 2 or more persons with CAH. After Taq I digestion, apparent deletions are seen in 7 of the 20 alleles of the probands; using EcoRI, apparent deletions are seen in 9 of the 20 alleles. However, the apparently deleted alleles seen with Taq I do not coincide with those seen with EcoRI. Furthermore, studies with Bgl II, EcoRI, Kpn I, and Xba I yield normal patterns with at least two enzymes in all cases. Since all probands yielded normal patterns with at least two of the five enzymes used, they conclude that the P450XXIA2 gene deletions widely reported in CAH patients probably represent gene conversions, unequal crossovers,or polymorphisms rather than simple gene deletions

  16. Early one-stage surgical reconstruction of the extremely high vagina in patients with congenital adrenal hyperplasia.

    Science.gov (United States)

    Donahoe, P K; Gustafson, M L

    1994-02-01

    High vaginal atresia is a very rare anomaly seen in the most severely masculinized females with congenital adrenal hyperplasia. These children have a foreshortened vagina conjoining the urogenital sinus proximal to the external urethral sphincter. In the past, they have undergone early clitoral recession and labioscrotal reduction, followed by vaginal pull-through at 2 to 4 years of age. Cumulative experience with repair of this anomaly has led us to attempt earlier one-stage intervention and to develop techniques that circumvent previously encountered vaginal stenoses. One-stage reconstruction of three older children (ages 2 to 9 years) involved: closure of the urethrovaginal fistula, mobilization of the vagina from the rectum and urethra, use of bilateral buttock flaps to augment the anterior vaginal wall, augmentation of the posterior wall with an inverted perineal U flap, clitoral recession, and advancement of labioscrotal and clitoral shaft flaps inferiorly to create labia majora and minora (respectively). The introiti were quite capacious after employing such flaps, did not require postoperative dilatation, and were free of strictures or urethrovaginal fistulae during long-term follow-up. Three younger patients were seen for initial evaluation at 8 to 12 months of age, when early one-stage reconstruction was undertaken. Paradoxically, these repairs were technically less difficult and did not require buttock flap augmentation because an island of anterior perineal skin could be rotated in to reach the anterior vaginal wall. A nerve stimulator was used to identify the external urethral sphincter, while the vagina was aggressively mobilized and advanced forward beyond the site of fistula closure on the urethra to avert formation of a urethro-vaginal fistula.2 +

  17. Massive neonatal adrenal enlargement due to cytomegaly, persistence of the transient cortex, and hyperplasia of the permanent cortex: findings in Cushing syndrome associated with hemihypertrophy.

    Science.gov (United States)

    Carney, J Aidan; Ho, Josephine; Kitsuda, Kazuteru; Young, William F; Stratakis, Constantine A

    2012-10-01

    Described in this article is the massive enlargement of both adrenal glands in 3 newborns-2 girls and 1 boy. Two had hemihypertrophy and other congenital abnormalities but no identified genetic mutation; the third had genetically proven Beckwith-Wiedemann syndrome. Two had severe Cushing syndrome, the third had hypercortisolemia but no clinical Cushing syndrome. Bilateral adrenalectomy cured Cushing syndrome in the 2 with severe symptoms; total adrenal weight in these patients was 44 and 53 g, respectively. Unilateral adrenalectomy was performed in the third patient: the gland weighed 52 g; postoperatively, the patient's hypercortisolemia normalized, and, concomitantly, the enlarged contralateral adrenal gland had a 5-fold decrease in size with slight enlargement 6 years postoperatively. Microscopically, the 3 patients had similar pathology: massive adrenal enlargement due to a combination of cytomegaly, persistence of the transient cortex, and hyperplasia of the permanent cortex. The pathologic findings were most likely the result of the genetic mutation identified in 1 patient and of an unknown mutation in the remaining 2 patients.

  18. Aberrant expression of glucagon receptors in adrenal glands of a patient with Cushing's syndrome and ACTH-independent macronodular adrenal hyperplasia Expresion aberrante de receptores de glucagón en tejido adrenal de un paciente con síndrome de Cushing e hiperplasia adrenal macronodular indedependiente de ACTH

    Directory of Open Access Journals (Sweden)

    Valeria de Miguel

    2010-06-01

    Full Text Available Adrenocorticotropin (ACTH independent bilateral macronodular adrenal hyperplasia (AIMAH is a rare cause of Cushing´s syndrome, characterized by bilateral adrenal lesions and excess cortisol production despite ACTH suppression. Cortisol synthesis is produced in response to abnormal activation of G-protein- coupled receptors, such as gastric inhibitory peptide, vasopressin, beta adrenergic agonists, LH/hCG and serotonin receptors. The aim of this study was to analyze the expression of glucagon receptors in adrenal glands from an AIMAH patient. A patient with ACTH-independent Cushing´s syndrome and bilateral macronodular adrenal hyperplasia was screened for altered activation of adrenal receptors by physiological (mixed meal and pharmacological (gonadotrophin releasing hormone, ACTH and glucagon tests. The results showed abnormally high levels of serum cortisol after stimulation with glucagon. Hypercortisolism was successfully managed with ketoconazole treatment. Interestingly, a 4-month treatment with a somatostatin analogue (octreotide was also able to reduce cortisol secretion. Finally, Cushing's syndrome was cured after bilateral adrenalectomy. Abnormal mRNA expression for glucagon receptor in the patient´s adrenal glands was observed by Real-Time PCR procedure. These results strongly suggest that the mechanism of AIMAH causing Cushing´s syndrome in this case involves the illicit activation of adrenal glucagon receptors. This is the first case reported of AIMAH associated with ectopic glucagon receptors.La hiperplasia adrenal macronodular bilateral independiente de ACTH (HAMIA es una causa infrecuente de Síndrome de Cushing, caracterizada por lesiones adrenales bilaterales, hipercortisolismo y ACTH plasmática suprimida. La síntesis de cortisol estaría regulada a través de ligandos de receptores asociados a proteína G que se expresan en forma aberrante en la corteza de las glándulas adrenales. El objetivo de este trabajo es analizar

  19. Congenital Adrenal Hyperplasia due to 17-alpha-hydoxylase/17,20-lyase Deficiency Presenting with Hypertension and Pseudohermaphroditism: First Case Report from Oman

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    Waad-Allah S. Mula-Abed

    2014-01-01

    Full Text Available This is the first report of congenital adrenal hyperplasia (CAH due to combined 17α-hydroxylase/17,20 lyase deficiency in an Omani patient who was initially treated for many years as a case of hypertension. CAH is an uncommon disorder that results from a defect in steroid hormones biosynthesis in the adrenal cortex. The clinical presentation depends on the site of enzymatic mutations and the types of accumulated steroid precursors. A 22-year-old woman who was diagnosed to have hypertension since the age of 10 years who was treated with anti-hypertensive therapy was referred to the National Diabetes and Endocrine Centre, Royal Hospital, Oman. The patient also had primary amenorrhea and features of sexual infantilism. Full laboratory and radio-imaging investigations were done. Adrenal steroids, pituitary function and karyotyping study were performed and the diagnosis was confirmed by molecular mutation study. Laboratory investigations revealed adrenal steroids and pituitary hormones profile in addition to 46XY karyotype that are consistent with the diagnosis of CAH due to 17α-hydroxylase deficiency. Extensive laboratory workup revealed low levels of serum cortisol (and its precursors 17α-hydroxyprogesterone and 11-deoxycortisol, adrenal androgens (dehydroepiandrosterone sulfate and androstenedione, and estrogen (estradiol; and high levels of mineralocorticoids precursors (11-deoxycorticosterone and corticosterone with high levels of ACTH, FSH and LH. Mutation analysis revealed CYP17A1-homozygous mutation (c.287G>A p.Arg96Gln resulting in the complete absence of 17α-hydroxylase/17,20-lyase activity. The patient was treated with dexamethasone and ethinyl estradiol with cessation of anti-hypertensive therapy. A review of the literature was conducted to identify previous studies related to this subtype of CAH. This is the first biochemically and genetically proven case of CAH due to 17α-hydroxylase/17,20-lyase deficiency in Oman and in the Arab

  20. A rare case of unilateral adrenal hyperplasia accompanied by hypokalaemic periodic paralysis caused by a novel dominant mutation in CACNA1S: features and prognosis after adrenalectomy.

    Science.gov (United States)

    Yang, Bo; Yang, Yuan; Tu, Wenling; Shen, Ying; Dong, Qiang

    2014-11-28

    Acute hypokalaemic paralysis is characterised by acute flaccid muscle weakness and has a complex aetiological spectrum. Herein we report, for the first time, a case of unilateral adrenal hyperplasia accompanied by hypokalaemic periodic paralysis type I resulting from a novel dominant mutation in CACNA1S. We present the clinical features and prognosis after adrenalectomy in this case. A 43-year-old Han Chinese male presented with severe hypokalaemic paralysis that remitted after taking oral potassium. The patient had suffered from periodic attacks of hypokalaemic paralysis for more than 20 years. A computed tomography (CT) scan of the abdomen showed a nodular mass on the left adrenal gland, although laboratory examination revealed the patient had not developed primary aldosteronism. The patient underwent a left adrenalectomy 4 days after admission, and the pathological examination further confirmed a 1.1 cm benign nodule at the periphery of the adrenal gland. Three months after the adrenalectomy, a paralytic attack recurred and the patient asked for assistance from the Department of Medical Genetics. His family history showed that two uncles, one brother, and a nephew also had a history of periodic paralysis, although their symptoms were milder. The patient's CACNA1S and SCN4A genes were sequenced, and a novel missense mutation, c.1582C > T (p.Arg528Cys), in CACNA1S was detected. Detection of the mutation in five adult male family members, including three with periodic paralysis and two with no history of the disease, indicated that this mutation caused hypokalaemic periodic paralysis type I in his family. Follow-up 2 years after adrenalectomy showed that the serum potassium concentration was increased between paralyses and the number and severity of paralytic attacks were significantly decreased. We identified a novel dominant mutation, c.1582C > T (p.Arg528Cys), in CACNA1S that causes hypokalaemic periodic paralysis. The therapeutic effect of adrenalectomy

  1. [Congenital adrenal hyperplasia due to lack of 17α-hydroxylase: a report of a new mutation in the gene CYP17A1].

    Science.gov (United States)

    Perales Martínez, J I; Pina Marqués, B; de Arriba Muñoz, A; Mayayo Dehesa, E; Labarta Aizpún, J I; Loidi Fernández, L

    2015-01-01

    P450c17 enzyme catalyses two different reactions: the 17α-hydroxylation of progesterone and pregnenolone, and segmenting the carbon 17-20 binding from the 17,20lyase producing adrenal androgens. This enzyme is coded by the CYP17A1 gene. The case is presented of a 14 year old patient with delayed pubertal development and a high blood pressure for height and age. 46,XX karyotype. Hormonal studies highlighted hypergonadotropic hypogonadism, adrenal insufficiency and mineralocorticoid excess. Subsequent genetic studies showed a homozygous mutation in the CYP17A1 gene (c.753+G>A), not previously described, which is responsible for the pathophysiology of 17α-hydroxylase deficiency. This entity is a rare form of congenital adrenal hyperplasia. The disease often goes unnoticed until adolescence or early adult life, and should be suspected in 46,XY individuals with ambiguous genitalia or 46,XX with delayed puberty associated with hypertension and/or hypokalaemia. Copyright © 2013 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.

  2. Pure Androgen-Secreting Adrenal Adenoma Associated with Resistant Hypertension

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    René Rodríguez-Gutiérrez

    2013-01-01

    Full Text Available Pure androgen-secreting adrenal adenoma is very rare, and its diagnosis remains a clinical challenge. Its association with resistant hypertension is uncommon and not well understood. We present an 18-year-old female with a 10-year history of hirsutism that was accidentally diagnosed with an adrenal mass during the evaluation of a hypertensive crisis. She had a long-standing history of hirsutism, clitorimegaly, deepening of the voice, and primary amenorrhea. She was phenotypically and socially a male. FSH, LH, prolactin, estradiol, 17-hydroxyprogesterone, and progesterone were normal. Total testosterone and DHEA-S were elevated. Cushing syndrome, primary aldosteronism, pheochromocytoma, and nonclassic congenital adrenal hyperplasia were ruled out. She underwent adrenalectomy and pathology reported an adenoma. At 2-month followup, hirsutism and virilizing symptoms clearly improved and blood pressure normalized without antihypertensive medications, current literature of this unusual illness and it association with hypertension is presented and discussed.

  3. Effect of large doses of 131I-19-iodocholesterol on metapyralone-induced adrenal cortical hyperplasia in dogs

    International Nuclear Information System (INIS)

    Anderson, B.G.; Beierwaltes, W.H.; Nishiyama, R.H.; Ice, R.D.

    1975-01-01

    The potential use of 131 I-19-iodocholesterol to treat ACTH excess Cushing's disease was evaluated in the dog. Three normal female dogs were given LD 50 radiation doses of 131 I-19-iodocholesterol without producing gross or histopathologically demonstrable change of the adrenals at autopsy 3 months later. The adrenal cortices of three dogs were made hyperplastic (to simulate the adrenal cortex in Cushing's disease) with ACTH and three with Metapyralone. In addition these six dogs were given LD 50 doses of 131 I-19-iodocholesterol. Three months after treatment, the adrenal glands of the ACTH-treated dogs were not enlarged, the cortex was thicker than normal, and there were no changes attributable to irradiation. At 3 months, the Metapyralone-treated dogs had enlarged adrenals, widening of the adrenal cortex, and no necrosis or other changes attributable to irradiation. It is concluded that a therapeutic trial of 131 I-19-iodocholesterol in the treatment of Cushing's disease is not indicated. (auth)

  4. Assembling a Functional Clitoris and Vulva from a Pseudo-Penis: A Surgical Technique for an Adult Woman with Congenital Adrenal Hyperplasia.

    Science.gov (United States)

    Tjalma, Wiebren A A

    2017-06-01

    Congenital adrenal hyperplasia (CAH) is associated with a genital deformation that might cause a negative body image. The genital ambiguity is generally "corrected" surgically during early infancy. The advantage is a psychological benefit. The disadvantages are multiple surgical procedures and the loss of orgasm. A 22-year-old woman with CAH consulted for genital reconstructive surgery. She had a pseudopenis of 4 cm and could achieve an orgasm by masturbating. During surgery, the penis was dismantled and with the preserved glans penis and the corpora cavernosa, a clitoris and vestibules were constructed, respectively. On the basis of the anamneses during the follow-up, she had a functional vagina and could still achieve orgasms. Genital correction surgery for CAH at an older age was easier, could be done in 1 step, and enabled the preservation of orgasm. Copyright © 2016 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

  5. Review of Outcome Information in 46,XX Patients with Congenital Adrenal Hyperplasia Assigned/Reared Male: What Does It Say about Gender Assignment?

    Directory of Open Access Journals (Sweden)

    Lee PeterA

    2010-12-01

    Full Text Available There is ample historical verification of 46,XX congenital adrenal hyperplasia (CAH patients being born with essentially male genitaliawhile outcome information is scant. Prior to glucocorticoid therapy, most patients died very young from adrenal insufficiency. Most available reports from laterchildhood, contain little information concerning sexual identity. Reports on older individuals lack adequate information about sexual identity and quality of life. The difficulty in assessing the relative impact of multiple dynamic environmental factors on the development of sexual identity, self- and body esteem and overall adjustment to life is clear. Nevertheless, it remains unclear whether those infants whose masculine genitalia at birth resulted in an initial male assignment would have enjoyed a better adult outcome had they been allowed to remain male rather than the female reassignment that most received. Further, one could ask whether a male sex of rearing should be considered in 46,XX CAH infants with male external genitalia. After reviewing available literature, we conclude that because those extremely virlized 46,XX CAH patients who were reared male with healthy social support demonstrated satisfactory levels of social and sexual function as adults a male sex assignment should be considered in these types of infants when social and cultural environment are supportive.

  6. Normal sex differences in prenatal growth and abnormal prenatal growth retardation associated with 46,XY disorders of sex development are absent in newborns with congenital adrenal hyperplasia due to 21-hydroxylase deficiency

    Directory of Open Access Journals (Sweden)

    Chalmers Laura J

    2011-05-01

    Full Text Available Abstract Background Congenital adrenal hyperplasia due to 21-hydroxylase deficiency is the most common presentation of a disorder of sex development (DSD in genetic females. A report of prenatal growth retardation in cases of 46,XY DSD, coupled with observations of below-optimal final height in both males and females with congenital adrenal hyperplasia due to 21-hydroxylase deficiency, prompted us to investigate prenatal growth in the latter group. Additionally, because girls with congenital adrenal hyperplasia are exposed to increased levels of androgens in the absence of a male sex-chromosome complement, the presence or absence of typical sex differences in growth of newborns would support or refute a hormonal explanation for these differences. Methods In total, 105 newborns with congenital adrenal hyperplasia were identified in our database. Gestational age (weeks, birth weight (kg, birth length (cm and parental heights (cm were obtained. Mid-parental height was considered in the analyses. Results Mean birth weight percentile for congenital adrenal hyperplasia was 49.26%, indicating no evidence of a difference in birth weight from the expected standard population median of 50th percentile (P > 0.05. The expected sex difference in favor of heavier males was not seen (P > 0.05. Of the 105 subjects, 44 (27%; 34 females, 10 males had birth length and gestational age recorded in their medical chart. Mean birth length for this subgroup was 50.90 cm (63rd percentile, which differed from the expected standard population median of 50th percentile (P = 0.0082. The expected sex difference in favor of longer males was also not seen (P > 0.05. Conclusion The prenatal growth retardation patterns reported in cases of 46,XY disorders of sex development do not generalize to people with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Sex differences in body weight and length typically seen in young infants were not seen in the subjects who

  7. Gonadal development and growth in 46,XX and 46,XY individuals with P450scc deficiency (congenital lipoid adrenal hyperplasia)

    DEFF Research Database (Denmark)

    Müller, J; Torsson, A; Damkjaer Nielsen, M

    1991-01-01

    and 8 years). In the surviving individuals, the diagnosis was established during the first 2-4 months of life by extensive endocrine studies of blood and urine. In the remaining patient, the diagnosis was made on the basis of karyotype (46,XY), anatomy of internal and external genitalia and adrenal...... normal breast and pubic hair development after oral estrogen replacement and topical testosterone administration. The glucocorticoid and mineralocorticoid replacement was adjusted in accordance with repeated measurements of serum sodium and serum potassium, plasma renin concentration and blood pressure...

  8. Single-Session CT-Guided Percutaneous Microwave Ablation of Bilateral Adrenal Gland Hyperplasia Due to Ectopic ACTH Syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Sarma, Asha, E-mail: ashasarma@gmail.com; Shyn, Paul B., E-mail: pshyn@partners.org [Brigham and Women’s Hospital, Department of Radiology (United States); Vivian, Mark A. [University of Manitoba, Department of Radiology (Canada); Ng, Ju-Mei [Brigham and Women’s Hospital, Department of Anesthesiology (United States); Tuncali, Kemal [Brigham and Women’s Hospital, Department of Radiology (United States); Lorch, Jorchen H. [Dana Farber Cancer Institute, Department of Medicine (United States); Zaheer, Sarah N.; Gordon, Michael S. [Brigham and Women’s Hospital, Department of Endocrinology (United States); Silverman, Stuart G. [Brigham and Women’s Hospital, Department of Radiology (United States)

    2015-10-15

    Bilateral adrenalectomy is currently the only available treatment for adrenocorticotropic hormone (ACTH)-dependent Cushing’s syndrome (ectopic ACTH syndrome) that is refractory to pharmacologic therapy. We describe two patients with refractory ectopic ACTH syndrome who were treated with CT-guided percutaneous microwave ablation of both hyperplastic adrenal glands in a single session: One was not a surgical candidate, and the other had undergone unsuccessful surgery. Following the procedure, both patients achieved substantial decreases in serum cortisol, symptomatic improvement, and decreased anti-hypertensive medication requirements.

  9. ARMC5 mutations in a large French-Canadian family with cortisol-secreting β-adrenergic/vasopressin responsive bilateral macronodular adrenal hyperplasia.

    Science.gov (United States)

    Bourdeau, Isabelle; Oble, Sylvie; Magne, Fabien; Lévesque, Isabelle; Cáceres-Gorriti, Katia Y; Nolet, Serge; Awadalla, Philip; Tremblay, Johanne; Hamet, Pavel; Fragoso, Maria Candida Barisson Villares; Lacroix, André

    2016-01-01

    Bilateral macronodular adrenal hyperplasia (BMAH) is a rare cause of Cushing's syndrome (CS) and its familial clustering has been described previously. Recent studies identified that ARMC5 mutations occur frequently in BMAH, but the relation between ARMC5 mutation and the expression of aberrant G-protein-coupled receptor has not been examined in detail yet. We studied a large French-Canadian family with BMAH and sub-clinical or overt CS. Screening was performed using the 1-mg dexamethasone suppression test (DST) in 28 family members. Screening for aberrant regulation of cortisol by various hormone receptors were examined in vivo in nine individuals. Sequencing of the coding regions of ARMC5 gene was carried out. Morning ambulating cortisol post 1 mg DST were >50 nmol/l in 5/8 members in generation II (57-68 years old), 9/22 in generation III (26-46 years old). Adrenal size was enlarged at different degrees. All affected patients increased cortisol following upright posture, insulin-induced hypoglycemia and/or isoproterenol infusion. β-blockers led to the reduction of cortisol secretion in all patients with the exception of two who had adrenalectomies because of β-blockers intolerance. We identified a heterozygous germline variant in the ARMC5 gene c.327_328insC, (p.Ala110Argfs*9) in nine individuals with clinical or subclinical CS, in four out of six individuals with abnormal suppression to dexamethasone at initial investigation and one out of six individuals with current normal clinical screening tests. Systematic screening of members of the same family with hereditary BMAH allows the diagnosis of unsuspected subclinical CS associated with early BMAH. The relation between the causative ARMC5 mutation and the reproducible pattern of aberrant β-adrenergic and V1-vasopressin receptors identified in this family remains to be elucidated. © 2016 European Society of Endocrinology.

  10. Health-Related Quality of Life, Mental Health and Psychotherapeutic Considerations for Women Diagnosed with a Disorder of Sexual Development: Congenital Adrenal Hyperplasia

    Directory of Open Access Journals (Sweden)

    Lindsey M. Brooks

    2010-01-01

    Full Text Available Little is known about the long-term health-related quality of life (HRQL and mental health outcomes for women diagnosed with congenital adrenal hyperplasia (CAH, a disorder of sex development. Though recommendations for therapists exist, no research has empirically investigated women's experiences in therapy or their recommendations for therapy. Thus the purpose of the study was to investigate HRQL, mental health concerns of women with CAH, and patients' recommendations for therapists. A qualitative methodology, consensual qualitative research, was used to answer these questions. Eight women with CAH participated in the study. Results from their interviews revealed six domains: physical functioning, psychological functioning, interpersonal processes and relationships, healthcare experiences of women with CAH, recommendations for health care professionals, and systemic considerations. Domains were further broken down into categories with results yielding implications for both medical and mental health professionals and shedding light on issues related to normalcy, concerns both related and unrelated to CAH, sex, and gender, and highlighting effective supports and therapeutic interventions.

  11. The Role of Support Groups, Advocacy Groups,andOther Interested Parties in Improving the Care of Patients with Congenital Adrenal Hyperplasia: Pleas and Warnings

    Directory of Open Access Journals (Sweden)

    Lee PeterA

    2010-05-01

    Full Text Available In the era of advocacy groups, it seems appropriate to contemplate how best to utilize them for patient benefit in the management of those with disorders of sex development (DSD, including those with congenital adrenal hyperplasia (CAH. Such interactions, to be constructive, require a spirit of cooperation to optimize outcomes. A traditional view of advocacy groups as a type of defender of patients' rights appears outdated and it is time that the benefits of their participation be fully realized. Open dialogue with all patients/families, including those who feel harmed by prior care are paramount. We discuss several recent examples of interactions that illustrate how dialogue in the name of "advocacy" can have a negative impact on developing a framework for ongoing constructive dialogue and actions. Such approaches completely change the dynamics of subsequent interactions. Physicians involved in the care of individuals with DSD, including those with CAH, and patients should be aware of confrontational techniques and legal implications that may be used by some advocacy groups. Hopefully recent efforts to promote a multidisciplinary care approach for patients with DSD/CAH will continue to foster mutual cooperation between team members, where the common goal is improving patient/family outcomes and quality of life.

  12. The Role of Support Groups, Advocacy Groups, and Other Interested Parties in Improving the Care of Patients with Congenital Adrenal Hyperplasia: Pleas and Warnings

    Directory of Open Access Journals (Sweden)

    Christopher P. Houk

    2010-01-01

    Full Text Available In the era of advocacy groups, it seems appropriate to contemplate how best to utilize them for patient benefit in the management of those with disorders of sex development (DSD, including those with congenital adrenal hyperplasia (CAH. Such interactions, to be constructive, require a spirit of cooperation to optimize outcomes. A traditional view of advocacy groups as a type of defender of patients' rights appears outdated and it is time that the benefits of their participation be fully realized. Open dialogue with all patients/families, including those who feel harmed by prior care are paramount. We discuss several recent examples of interactions that illustrate how dialogue in the name of “advocacy” can have a negative impact on developing a framework for ongoing constructive dialogue and actions. Such approaches completely change the dynamics of subsequent interactions. Physicians involved in the care of individuals with DSD, including those with CAH, and patients should be aware of confrontational techniques and legal implications that may be used by some advocacy groups. Hopefully recent efforts to promote a multidisciplinary care approach for patients with DSD/CAH will continue to foster mutual cooperation between team members, where the common goal is improving patient/family outcomes and quality of life.

  13. Mental Health and Disorders of Sex Development/Intersex Conditions in Iranian Culture: Congenital Adrenal Hyperplasia, 5-α Reductase Deficiency-Type 2, and Complete Androgen Insensitivity Syndrome.

    Science.gov (United States)

    Khorashad, Behzad S; Aghili, Zahra; Kreukels, Baudewijntje P C; Reid, Alistair G; Roshan, Ghasem M; Hiradfar, Mehran; Talaei, Ali; Cohen Kettenis, Peggy T

    2018-05-01

    Sixty-one patients (22 patients with congenital adrenal hyperplasia [CAH] with a mean age of 14.86 years [range, 5-23], 20 patients with 5-α reductase deficiency type 2 [5α-RD-2] with a mean age of 19.5 years [range, 5-29], and 19 patients with complete androgen insensitivity syndrome [CAIS] with a mean age of 18.26 years [range, 5-28]) were evaluated using the Kiddie Schedule for Affective Disorders and Schizophrenia, the Structured Clinical Interview for DSM-IV Axis I, Axis II, and the Global Assessment Functioning Scale. All participants were female-assigned at birth. Ten patients (16.4%) transitioned to the male gender. Overall, 68% of patients had one or more lifetime Axis I disorders, including 63.6% of the CAH participants, 90% of 5α-RD-2 participants, and 52.6% of the CAIS participants. The most commonly observed were affective disorders (27.9%), gender identity disorder (27.9%), and anxiety (16.4%). Our study demonstrates that mental health of Iranian patients with DSD is at risk. This might be due to the fact that patients with DSD conditions are mostly treated medically and their mental health is often superficially addressed in developing countries such as Iran, at least in the past. We argue that it is important to pay attention to the mental health issues of patients with DSD and focus on specific issues, which may vary cross-culturally.

  14. Obesity in children with congenital adrenal hyperplasia in the Minnesota cohort: importance of adjusting body mass index for height-age.

    Science.gov (United States)

    Sarafoglou, Kyriakie; Forlenza, Gregory P; Yaw Addo, O; Kyllo, Jennifer; Lteif, Aida; Hindmarsh, P C; Petryk, Anna; Gonzalez-Bolanos, Maria Teresa; Miller, Bradley S; Thomas, William

    2017-05-01

    To evaluate obesity and overweight in children with congenital adrenal hyperplasia (CAH) and associations with glucocorticoids, fludrocortisone and disease control. Adjusting body mass index-for-height-age (BMI HA ) percentile is proposed to correct misclassification of obese/overweight status in CAH children with advanced bone age and tall-for-age stature. Longitudinal. One hundred and ninety-four children with CAH seen from 1970 to 2013: 124 salt wasting (SW); 70 simple virilizing (SV); 102 females. Body mass index (BMI) end-points were overweight (85-94 percentile) and obese (≥95 percentile). Approximately 50% of the children had at least one BMI measurement ≥95 percentile and about 70% had at least one ≥85 percentile. Using BMI HA percentiles, obesity incidence decreased slightly in SW children (47-43%) and markedly in SV children (50-33%); however, overweight status was not reduced. Only 6% of SW and 1% of SV children were persistently obese (≥3 clinic visits) when BMI HA was applied, whereas overweight status persisted in 35% of SW and 33% of SV children. Most obesity or overweight when using BMI HA occurred before age 10 and there was no association with hydrocortisone (HC) or fludrocortisone dosing. Adiposity rebound for SW children occurred by 3·3 years and in SV females by age 3·8 years, over a year earlier than the adiposity rebound for healthy children. Children with CAH are at higher risk for early onset obesity and overweight with or without using BMI HA but rates of persistent obesity were lower than previously reported. Careful HC dosing during early childhood is needed to prevent increased weight gain and an early adiposity rebound. © 2017 John Wiley & Sons Ltd.

  15. Clinical profile and inheritance pattern of CYP21A2 gene mutations in patients with classical congenital adrenal hyperplasia from 10 families

    Directory of Open Access Journals (Sweden)

    Sarita Yadav

    2015-01-01

    Full Text Available Context: Congenital adrenal hyperplasia (CAH is an autosomal recessive metabolic disorder caused by mutations in the CYP21A2 gene. Genetic diagnosis of 21-OH deficiency causing CAH is more complicated than any other monogenic disorder due to high variability of the locus. The disease has a wide spectrum of clinical variants making it difficult to establish a genotype-phenotype correlation. Therefore, family studies are necessary to ascertain parental genotype and segregation of the mutant allele among the offspring. Aim: The present study aimed to identify CYP21A2 gene mutations and analyze the segregation pattern in CAH trios (patients and their parents. Materials and Methods: A total of ten families having at least one CAH child were recruited. Results: Out of 31 children from ten families, 15 were affected with CAH and 13 of /them (12 females and 1 male were available for genetic testing. One family had all the children affected with CAH. Compound heterozygous mutations were identified in seven patients (53.8% whereas p.P30L, In2 and δ8 bp mutations were present in homozygous state in three (23.1%, two (15.3 % and one (7.6% patient respectively. Conclusions: In majority of the families, mutant alleles observed in the patients were inherited from the parents whereas three families showed sporadic mutations without any paternal or maternal origin. This indicated their novel occurrence due to misalignment of the parental genes and /or large deletion of the gene. Female preponderance was noted in the CAH families and also among the patients raising the possibility of survival advantage among females.

  16. A Novel Mutation in the CYP11B1 Gene Causes Steroid 11β-Hydroxylase Deficient Congenital Adrenal Hyperplasia with Reversible Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Mohammad A. Alqahtani

    2015-01-01

    Full Text Available Congenital adrenal hyperplasia (CAH due to steroid 11β-hydroxylase deficiency is the second most common form of CAH, resulting from a mutation in the CYP11B1 gene. Steroid 11β-hydroxylase deficiency results in excessive mineralcorticoids and androgen production leading to hypertension, precocious puberty with acne, enlarged penis, and hyperpigmentation of scrotum of genetically male infants. In the present study, we reported 3 male cases from a Saudi family who presented with penile enlargement, progressive darkness of skin, hypertension, and cardiomyopathy. The elder patient died due to heart failure and his younger brothers were treated with hydrocortisone and antihypertensive medications. Six months following treatment, cardiomyopathy disappeared with normal blood pressure and improvement in the skin pigmentation. The underlying molecular defect was investigated by PCR-sequencing analysis of all coding exons and intron-exon boundary of the CYP11B1 gene. A novel biallelic mutation c.780 G>A in exon 4 of the CYP11B1 gene was found in the patients. The mutation created a premature stop codon at amino acid 260 (p.W260∗, resulting in a truncated protein devoid of 11β-hydroxylase activity. Interestingly, a somatic mutation at the same codon (c.779 G>A, p.W260∗ was reported in a patient with papillary thyroid cancer (COSMIC database. In conclusion, we have identified a novel nonsense mutation in the CYP11B1 gene that causes classic steroid 11β-hydroxylase deficient CAH. Cardiomyopathy and cardiac failure can be reversed by early diagnosis and treatment.

  17. Endometrial Hyperplasia

    Science.gov (United States)

    ... Patients Search FAQs Endometrial Hyperplasia Page Navigation ▼ ACOG Pregnancy Book Endometrial Hyperplasia Patient Education FAQs Endometrial Hyperplasia Patient Education Pamphlets - Spanish Endometrial ...

  18. A new compound heterozygous frameshift mutation in the type II 3{beta}-hydroxysteroid dehydrogenase 3{beta}-HSD gene causes salt-wasting 3{beta}-HSD deficiency congenital adrenal hyperplasia

    Energy Technology Data Exchange (ETDEWEB)

    Zhang, L.; Sakkal-Alkaddour, S.; Chang, Ying T.; Yang, Xiaojiang; Songya Pang [Univ. of Illinois, Chicago, IL (United States)

    1996-01-01

    We report a new compound heterozygous frameshift mutation in the type II 3{Beta}-hydroxysteroid dehydrogenase (3{beta}-HSD) gene in a Pakistanian female child with the salt-wasting form of 3{Beta}-HSD deficiency congenital adrenal hyperplasia. The etiology for her congenital adrenal hyperplasia was not defined. Although the family history suggested possible 3{beta}-HSd deficiency disorder, suppressed adrenal function caused by excess glucocorticoid therapy in this child at 7 yr of age did not allow hormonal diagnosis. To confirm 3{beta}-HSD deficiency, we sequenced the type II 3{beta}-HSD gene in the patient, her family, and the parents of her deceased paternal cousins. The type II 3{beta}-HSD gene region of a putative promotor, exons I, II, III, and IV, and exon-intron boundaries were amplified by PCR and sequenced in all subjects. The DNA sequence of the child revealed a single nucleotide deletion at codon 318 [ACA(Thr){r_arrow}AA] in exon IV in one allele, and two nucleotide deletions at codon 273 [AAA(Lys){r_arrow}A] in exon IV in the other allele. The remaining gene sequences were normal. The codon 318 mutation was found in one allele from the father, brother, and parents of the deceased paternal cousins. The codon 273 mutation was found in one allele of the mother and a sister. These findings confirmed inherited 3{beta}-HSD deficiency in the child caused by the compound heterozygous type II 3{beta}-HSD gene mutation. Both codons at codons 279 and 367, respectively, are predicted to result in an altered and truncated type II 3{beta}-HSD protein, thereby causing salt-wasting 3{beta}-HSD deficiency in the patient. 21 refs., 2 figs., 1 tab.

  19. Quantification of nonclassicality

    Energy Technology Data Exchange (ETDEWEB)

    Mraz, Melanie; Sperling, Jan; Vogel, Werner; Hage, Boris [Universitaet Rostock, Institut fuer Physik, Rostock (Germany)

    2013-07-01

    At the beginning of the 20th century the discussion on physics beyond the classical regime started. This was the hour of birth of quantum physics and, with Einstein's description of the photoelectric effect, of quantum optics. Even the physicists had problems to understand nonclassical quantum phenomena, because of its non-intuitive properties. So, why further struggling? Nonclassical states have an advantage over classical states for various applications. Only one example is the quantum teleportation which would be unthinkable without nonclassical states. Hence, it is of a fundamental interest to study properties of nonclassical quantum states. It is already possible to say if a state is nonclassical or not, but how can we decide how much nonclassicality is in our system? We propose a degree of nonclassicality being a nonclassicality measure. It is determined by the decomposition of a quantum state into superpositions of coherent states. On the one hand, coherent states resembles the behavior of a classical harmonic oscillator most closely. On the other hand, the more quantum superpositions of coherent states are needed, the more quantum interferences arise. A method for such a decomposition of quantum states is presented and the degree of nonclassicality is determined for different states. We apply our method to typical nonclassical states, such as the compass state and the squeezed vacuum state.

  20. Imaging findings of neonatal adrenal disorders

    International Nuclear Information System (INIS)

    Yoon, Hye Kyung; Han, Bo Kyung; Lee, Min Hee

    1999-01-01

    In newborn infants, normal adrenal glands are characterized by a relatively thin echogenic center surrounded by a thick, hypoechoic cortical rim as seen on ultrasound (US). Various disorders involving the neonatal adrenal gland include adrenal hemorrhage, hyperplasia, cyst, Wolman's disease, and congenital neuroblastoma. Adrenal hemorrhage is the most common cause of an adrenal mass in the neonate, though differentiation between adrenal hemorrhage and neuroblastoma is in many cases difficult. We describe characteristic US, CT and MR imaging findings in neonates with various adrenal disorders

  1. Imaging findings of neonatal adrenal disorders

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, Hye Kyung; Han, Bo Kyung; Lee, Min Hee [Sungkyunkwan Univ. College of Medicine, Seoul (Korea, Republic of)

    1999-01-01

    In newborn infants, normal adrenal glands are characterized by a relatively thin echogenic center surrounded by a thick, hypoechoic cortical rim as seen on ultrasound (US). Various disorders involving the neonatal adrenal gland include adrenal hemorrhage, hyperplasia, cyst, Wolman's disease, and congenital neuroblastoma. Adrenal hemorrhage is the most common cause of an adrenal mass in the neonate, though differentiation between adrenal hemorrhage and neuroblastoma is in many cases difficult. We describe characteristic US, CT and MR imaging findings in neonates with various adrenal disorders.

  2. Radiologic evaluation of adrenal glands

    International Nuclear Information System (INIS)

    Pradel, J.; Bruel, J.M.; Taourel, P.; Garnier, T.; Cyteval, C.; Lamarque, J.L.

    1990-01-01

    When a diagnosis of adrenal disorder is suspected on the basis of clinical manifestations and/or laboratory findings, computed tomography (CT) is generally accepted as the imaging procedure of choice for visualization of adrenal areas and localization of lesions. Sonography keeps an important role in discovering adrenal masses during investigation for other suspected abnormality (incidentaloma). 131 I MIBG scintigraphy provides an efficious mean of pheochromocytoma localization and functional characterization. These non invasive procedures have greatly reduced the need for arteriography and venography; adrenal venous sampling is still an useful method for localizing either a tumor or hyperplasia related to primary aldosteronism. MR imaging and CT are nearly equivalent in the detection of adrenal masses: besides MR imaging has a potential for characterization of adrenal masses which might be useful, especially in distinguishing adrenal adenomas from malignant neoplasms, obviating, in some cases, the need of CT guided adrenal biopsy [fr

  3. Hiperplasia adrenal congênita: dosagem da 17-hidroxiprogesterona basal para seleção e casos para realização do teste de estímulo com ACTH sintético Congenital adrenal hyperplasia: measurement of basal 17-hydroxyprogesterone as a screening test to select patients for the synthetic ACTH provocative test

    Directory of Open Access Journals (Sweden)

    Lúcia Helena Coelho Nóbrega

    2004-05-01

    Full Text Available OBJETIVOS: avaliar se os níveis de 17-hidroxiprogesterona podem predizer o resultado do teste de estímulo como diagnóstico de hiperplasia adrenal congênita, forma tardia. MÉTODOS: foram incluídas no estudo e avaliadas retrospectivamente 122 pacientes com suspeita clínica de hiperplasia adrenal congênita forma tardia. Essa suspeita clínica incluía sinais e/ou sintomas de hiperandrogenismo (hirsutismo, acne, pele oleosa, irregularidade menstrual, etc.. Todas as pacientes foram submetidas ao teste de estímulo da adrenal com ACTH sintético 0,25 mg (Synacthen®. Após repouso de 60 minutos as amostras foram colhidas nos tempos basal e 60 minutos após a administração de 0,25 mg de ACTH sintético para dosagem de 17-hidroxiprogesterona, sendo mantido o acesso venoso com catéter heparinizado. Foi utilizado o método de radioimunoensaio para realizar as dosagens séricas da 17-hidroxiprogesterona. A sensibilidade e a especificidade da 17-hidroxiprogesterona basal como teste de rastreamento para hiperplasia adrenal congênita foram medidas, avaliando vários pontos de corte. Curvas ROC foram feitas para analisar a performance do teste, utilizando o software Medcalc®. RESULTADOS: a análise por curva ROC mostrou um ponto de corte de 181 ng/dl acima do qual dever-se-ia realizar o teste de estímulo, bem próximo a 200 ng/dl, mais comumente aceito pela literatura. Níveis séricos da 17-hidroxiprogesterona mais altos que 200 ng/dl têm valores preditivo positivo e negativo de 75% e 100% e acurácia de 98,4% como diagnóstico de hiperplasia adrenal não-clássica. CONCLUSÕES: considerando os dados, sugerimos que pacientes com hiperandrogenismo clínico devam iniciar a investigação com 17-hidroxiprogesterona basal e, caso esta se mostre acima de 181 ng/dl, sigam a investigação com o teste de estímulo com ACTH sintético.INTRODUCTION: adrenal hyperplasia is a common genetic disorder and 95% of the cases are due to a 21-hydroxylase

  4. Determination of adrenal volume by MRI in healthy children

    DEFF Research Database (Denmark)

    Mouritsen, Annette; Johansen, Marie Lindhardt; Wohlfahrt-Veje, Christine

    2014-01-01

    BACKGROUND: Adrenal disorders such as congenital adrenal hyperplasia result in abnormal adrenal size and morphology, but little is known about the clinical value of magnetic resonance imaging (MRI) in determining adrenal volume. OBJECTIVE: To evaluate the potential usefulness of MR methodology...

  5. Hiperplasia supra-renal congênita por deficiência de 11-ß-hidroxilase Congenital adrenal hyperplasia due to 11-beta-hydroxylase deficiency

    Directory of Open Access Journals (Sweden)

    Ramires Tosatti Júnior

    2005-12-01

    Full Text Available Este artigo tem o objetivo de relatar o diagnóstico e a evolução clínica de um paciente de 15 anos portador de uma disfunção congênita da esteroideogênese adrenal, que pode apresentar-se como hipertensão arterial de diagnóstico muitas vezes tardio (adolescência, virilização ou formas perdedoras de sal (nascimento e infância.The objective of this article is to relate the diagnostic and clinical evolution of a 15 year old patient with a congenital adrenal steroidogenesis dysfunction that can present as hypertension diagnosed later in life (adolescence, virilization or salt wasting (birth and childhood.

  6. Reconstrucción genital integral en la Hiperplasia Suprarrenal Congénita: sensibilidad, estética y función (embarazo Integral genital reconstruction: sensitivity, aesthetic and function (pregnancy, in the Congenital Adrenal Hyperplasia

    Directory of Open Access Journals (Sweden)

    A.A. Núñez Serrano

    2010-03-01

    Full Text Available La Hiperplasia Suprarrenal Congénita o Síndrome Adrenogenital es un cuadro clínico poco frecuente. Las anomalías que presenta van asociadas a síndromes que a veces son muy complejos, destacando, entre otros, el seudohermafroditismo femenino y el hermafroditismo verdadero. Las mujeres que la padecen presentan como alteración más evidente un clítoris aumentado de tamaño (megaloclítoris y otras alteraciones en los genitales externos. Ante estas pacientes es necesario realizar un diagnostico correcto mediante exploración física y otras pruebas complementarias: estudios genéticos, analíticos, hematológicos y urinarios. La corrección quirúrgica del megaloclítoris es necesaria no sólo para mejorar el aspecto de los genitales externos, sino también para que tanto la paciente como su familia se encuentren menos afectados psicológicamente. Existen varias técnicas quirúrgicas que intentan corregir esta malformación, como la amputación o clitorectomía total, la retroposición o enterramiento y la clitorectomía parcial selectiva. Todas ellas, junto con otras correcciones de los genitales externos afectados, han sido utilizadas hasta el día de hoy. Presentamos el caso de una paciente con Hiperplasia Suprarrenal Congénita por déficit del enzima 21-hidroxilasa. Describimos su clínica, la técnica quirúrgica aplicada en su caso y, sobre todo, dado la rareza de esta patología, comunicamos que, tras dos embarazos, los dos hijos nacidos, no presentaron la alteración congénita materna.Congenital Adrenal Hyperplasia is a rare patology, whith clinical expressions like female pseudohermaphroditism or true hermaphroditism. Females affected mainly suffer clitoral hypertrophy and external genitalia abnormalityes. The complete diagnosis includes: careful examination of the genitals, complementary imaging proofs, hormonal and genetic testing. The surgical correction of the external genitals and megaloclitoris improves the physical and

  7. Increased Cross-Gender Identification Independent of Gender Role Behavior in Girls with Congenital Adrenal Hyperplasia: Results from a Standardized Assessment of 4- to 11-Year-Old Children.

    Science.gov (United States)

    Pasterski, Vickie; Zucker, Kenneth J; Hindmarsh, Peter C; Hughes, Ieuan A; Acerini, Carlo; Spencer, Debra; Neufeld, Sharon; Hines, Melissa

    2015-07-01

    While reports showing a link between prenatal androgen exposure and human gender role behavior are consistent and the effects are robust, associations to gender identity or cross-gender identification are less clear. The aim of the current study was to investigate potential cross-gender identification in girls exposed prenatally to high concentrations of androgens due to classical congenital adrenal hyperplasia (CAH). Assessment included two standardized measures and a short parent interview assessing frequency of behavioral features of cross-gender identification as conceptualized in Part A of the diagnostic criteria for gender identity disorder (GID) in the DSM-IV-TR. Next, because existing measures may have conflated gender role behavior with gender identity and because the distinction is potentially informative, we factor analyzed items from the measures which included both gender identity and gender role items to establish the independence of the two constructs. Participants were 43 girls and 38 boys with CAH and 41 unaffected female and 31 unaffected male relatives, aged 4- to 11-years. Girls with CAH had more cross-gender responses than female controls on all three measures of cross-gender identification as well as on a composite measure of gender identity independent of gender role behavior. Furthermore, parent report indicated that 5/39 (12.8 %) of the girls with CAH exhibited cross-gender behavior in all five behavioral domains which comprise the cross-gender identification component of GID compared to 0/105 (0.0 %) of the children in the other three groups combined. These data suggest that girls exposed to high concentrations of androgens prenatally are more likely to show cross-gender identification than girls without CAH or boys with and without CAH. Our findings suggest that prenatal androgen exposure could play a role in gender identity development in healthy children, and may be relevant to gender assignment in cases of prenatal hormone disruption

  8. Witnessing nonclassical multipartite states

    International Nuclear Information System (INIS)

    Saguia, A.; Rulli, C. C.; Oliveira, Thiago R. de; Sarandy, M. S.

    2011-01-01

    We investigate a witness for nonclassical multipartite states based on their disturbance under local measurements. The witness operator provides a sufficient condition for nonclassicality that coincides with a nonvanishing global quantum discord, but it does not demand an extremization procedure. Moreover, for the case of Z 2 -symmetric systems, we rewrite the witness in terms of correlation functions so that classicality is found to necessarily require either vanishing magnetization in the invariant axis or isotropy of the two-point function in the transverse spin plane. We illustrate our results in quantum spin chains, where a characterization of factorized ground states (with spontaneously broken Z 2 symmetry) is achieved. As a by-product, the witness is also shown to indicate a second-order quantum phase transitions, which is illustrated for both the XY and the Ashkin-Teller spin chains.

  9. Treatment outcomes in congenital adrenal hyperplasia.

    Science.gov (United States)

    Cheng, Tina Q; Speiser, Phyllis W

    2012-01-01

    Systematic literature review and meta-analysis guided by expert opinion has refined current approaches to the treatment of CAH. The advent of widespread newborn screening has improved outcomes, with lower morbidities and mortality. Future advances may be recognized in the form of more efficient diagnostic tools, physiologic drug delivery, improved surgical methods, and assisted reproductive technologies.

  10. Transgenic expression of nonclassically secreted FGF suppresses kidney repair.

    Directory of Open Access Journals (Sweden)

    Aleksandr Kirov

    Full Text Available FGF1 is a signal peptide-less nonclassically released growth factor that is involved in angiogenesis, tissue repair, inflammation, and carcinogenesis. The effects of nonclassical FGF export in vivo are not sufficiently studied. We produced transgenic mice expressing FGF1 in endothelial cells (EC, which allowed the detection of FGF1 export to the vasculature, and studied the efficiency of postischemic kidney repair in these animals. Although FGF1 transgenic mice had a normal phenotype with unperturbed kidney structure, they showed a severely inhibited kidney repair after unilateral ischemia/reperfusion. This was manifested by a strong decrease of postischemic kidney size and weight, whereas the undamaged contralateral kidney exhibited an enhanced compensatory size increase. In addition, the postischemic kidneys of transgenic mice were characterized by hyperplasia of interstitial cells, paucity of epithelial tubular structures, increase of the areas occupied by connective tissue, and neutrophil and macrophage infiltration. The continuous treatment of transgenic mice with the cell membrane stabilizer, taurine, inhibited nonclassical FGF1 export and significantly rescued postischemic kidney repair. It was also found that similar to EC, the transgenic expression of FGF1 in monocytes and macrophages suppresses kidney repair. We suggest that nonclassical export may be used as a target for the treatment of pathologies involving signal peptide-less FGFs.

  11. Optimal glucocorticoid replacement in adrenal insufficiency.

    Science.gov (United States)

    Øksnes, Marianne; Ross, Richard; Løvås, Kristian

    2015-01-01

    Adrenal insufficiency (glucocorticoid deficiency) comprises a group of rare diseases, including primary adrenal insufficiency, secondary adrenal insufficiency and congenital adrenal hyperplasia. Lifesaving glucocorticoid therapy was introduced over 60 years ago, but since then a number of advances in treatment have taken place. Specifically, little is known about short- and long-term treatment effects, and morbidity and mortality. Over the past decade, systematic cohort and registry studies have described reduced health-related quality of life, an unfavourable metabolic profile and increased mortality in patients with adrenal insufficiency, which may relate to unphysiological glucocorticoid replacement. This has led to the development of new modes of replacement that aim to mimic normal glucocorticoid physiology. Here, evidence for the inadequacy of conventional glucocorticoid therapy and recent developments in treatment are reviewed, with an emphasis on primary adrenal insufficiency. Copyright © 2014 Elsevier Ltd. All rights reserved.

  12. Study of awareness of adrenal disorders among interns and postgraduate students of Hamidia Hospital, Bhopal

    Directory of Open Access Journals (Sweden)

    Sachin Chittawar

    2017-01-01

    Full Text Available Introduction: Adrenal disorders could be a life-threatening emergency, hence requires immediate therapeutic management. For this awareness regarding its diagnosis, management, and treatment is prime important. Aims and Objective: To study the awareness of adrenal disorders among interns and postgraduates students of Hamidia Hospital, Bhopal. Materials and Methods: A cross-sectional questionnaire-based study was performed. Fifty-six participants, i.e., 1st, 2nd, and 3rd years postgraduate residents of general medicine (n = 14 × 3 and interns (n = 14 were included in the study. There were 12 questions on adrenal insufficiency, adrenal adenoma, congenital adrenal hyperplasia (CAH, nonclassical CAH (NCCAH, pheochromocytoma, and Conn's syndrome. One mark was awarded for each correct response. Results: In the present study, 14 (25% participants scored < 5 marks, 33 (58.9% scored between 6 and 9, and 9 (16.1% scored between 10 and 12. The mean score among the participants was 6.38 ± 2.505, with a range from 2 to 11 marks. The number of correct answers by postgraduates residents of 1st year was 101, 2nd year was 95, and 3rd year was 93 and interns scored 68 out of total 168 questions in each group. Mean awareness score for residents of 1st, 2nd, 3rd years participants and interns was 7.21 ± 2.806, 6.79 ± 2.119, and 6.64 ± 2.818 and 6.63 ± 2.505, respectively. Most of the participants recorded correct responses related to diagnosis (57.7% followed by responses related to treatment (64.3%. Answers to a question regarding how commonly is adrenal insufficiency diagnosed in medical Intensive Care Unit, none of the individuals responded correctly.Conclusion: There was a lack of awareness regarding diagnosis, management, and treatment of adrenal disorders in central India. We need to prioritize training related to these illnesses in our postgraduate teaching curriculum in practice.

  13. Adrenal scintigraphy

    International Nuclear Information System (INIS)

    Beierwaltes, W.H.

    1979-01-01

    The following items are discussed:anatomy and physiology of adrenal glands, clinical indications of scintigraphy, radiobiology and radiochemistry, scintigraphic imaging, adrenocortical hyperfunction, aldosteronism and hypertension associated with low renin level, excess of androgen, adrenocortical hyperfunction and future perspectives of adrenal scintigraphy. (M.A.) [pt

  14. Coexistence of Cushing syndrome from functional adrenal adenoma and Addison disease from immune-mediated adrenalitis.

    Science.gov (United States)

    Colucci, Randall; Jimenez, Rafael E; Farrar, William; Malgor, Ramiro; Kohn, Leonard; Schwartz, Frank L

    2012-06-01

    A 56-year-old woman presented with an incidental adrenal adenoma and physical examination findings that included moderate obesity, a slight cervicothoracic fat pad ("buffalo hump"), increased supraclavicular fat pads, and white abdominal striae. Biochemical workup revealed elevated levels of 24-hour urinary free cortisol but normal serum morning cortisol and suppressed levels of corticotropin, suggestive of adrenal-dependent Cushing syndrome. The resected adrenal gland revealed macronodular cortical hyperplasia with a dominant nodule. Other findings included an absent cortisol response to corticotropin stimulation, presence of serum anti-21-hydroxylase antibodies, and mononuclear cell infiltration--consistent with adrenalitis. The findings represent, to the authors' knowledge, the first known case of a patient with coexistent functional cortisol-secreting macronodular adrenal tumor resulting in Cushing syndrome and immune-mediated adrenalitis resulting in Addison disease.

  15. Adrenal scintigraphy

    International Nuclear Information System (INIS)

    Veen, E.A. van der.

    1978-01-01

    The visualization of functioning adrenocortical tissue by scintigraphy became possible with the introduction of radioiodinated cholesterol derivatives. According to the literature, there is evidence that one of these iodinated cholesterols, 6-β-iodomethyl-nor-cholesterol, concentrates in the adrenal cortex to a much greater extent than 131 I-19-odocholesterol. Results comparing both radiopharmaceuticals are described. The authors investigated the possibility of increasing the uptake of iodinated cholesterol using simultaneous ACTH and the 'cholesterol side-chain cleavage enzymeblocker': aminoglutethimide. The results of adrenal scintigraphy performed in 37 patients are described. Finally, the literature on adrenal scintigraphy is reviewed, and results reported in various studies are compared. (Auth.)

  16. Adrenal Insufficiency

    Science.gov (United States)

    ... proper balance of salt and water in the body. Regulates blood volume and blood pressure. Adrenal androgens Helps regulate pubic and armpit (weak male sex hormones hair growth in women. present in both sexes) Some causes may be ...

  17. Clinicopathological correlates of adrenal Cushing's syndrome.

    Science.gov (United States)

    Duan, Kai; Gomez Hernandez, Karen; Mete, Ozgur

    2015-03-01

    Endogenous Cushing's syndrome is a rare endocrine disorder that incurs significant cardiovascular morbidity and mortality, due to glucocorticoid excess. It comprises adrenal (20%) and non-adrenal (80%) aetiologies. While the majority of cases are attributed to pituitary or ectopic corticotropin (ACTH) overproduction, primary cortisol-producing adrenal cortical lesions are increasingly recognised in the pathophysiology of Cushing's syndrome. Our understanding of this disease has progressed substantially over the past decade. Recently, important mechanisms underlying the pathogenesis of adrenal hypercortisolism have been elucidated with the discovery of mutations in cyclic AMP signalling (PRKACA, PRKAR1A, GNAS, PDE11A, PDE8B), armadillo repeat containing 5 gene (ARMC5) a putative tumour suppressor gene, aberrant G-protein-coupled receptors, and intra-adrenal secretion of ACTH. Accurate subtyping of Cushing's syndrome is crucial for treatment decision-making and requires a complete integration of clinical, biochemical, imaging and pathology findings. Pathological correlates in the adrenal glands include hyperplasia, adenoma and carcinoma. While the most common presentation is diffuse adrenocortical hyperplasia secondary to excess ACTH production, this entity is usually treated with pituitary or ectopic tumour resection. Therefore, when confronted with adrenalectomy specimens in the setting of Cushing's syndrome, surgical pathologists are most commonly exposed to adrenocortical adenomas, carcinomas and primary macronodular or micronodular hyperplasia. This review provides an update on the rapidly evolving knowledge of adrenal Cushing's syndrome and discusses the clinicopathological correlations of this important disease. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

  18. Segmental arterial mediolysis with mesangial cell hyperplasia

    DEFF Research Database (Denmark)

    Slavin, Richard E.; Leifsson, Páll Skúli

    2017-01-01

    Background: Segmental arterial mediolysis (SAM) a rare arteriopathy causing massive bleeding or ischemic symptoms, is suspected of representing a vascular disease of the peripheral sympathetic nervous system. It is initiated by the supra physiological release of norepinephrine from the efferent...... by conditions causing the adrenal medulla to release supra physiologic levels of circulating norepinephrine. Supra physiologic release of norepinephrine from the peripheral sympathetic nerves also can cause mesangial hyperplasia that can be accompanied with segmental glomerular loop sclerosis-making it another...

  19. Preface: Nonclassical Transport

    International Nuclear Information System (INIS)

    Bolshov, L.; Kondratenko, P.; Pruess, K.

    2008-01-01

    Transport phenomena in highly heterogeneous media can be dramatically different from those in homogeneous media and therefore are of great fundamental and practical interest. Anomalous transport occurs in semiconductor physics, plasma physics, astrophysics, biology, and other areas. It plays an especially important role in hydrogeology because it may govern the rate of migration and degree of dispersion of groundwater contaminants from hazardous waste sites. The series of four articles in this special section of Vadose Zone Journal is devoted to transport phenomena in heterogeneous media in the context of geologic disposal of radioactive waste. It contains the results of joint investigations performed at the Nuclear Safety Institute of the Russian Academy of Sciences and Lawrence Berkeley National Laboratory in California. The work was supported by the U.S. DOE (under Contract No. DEAC02-05CH11231). The problems addressed in this research involve a broad range of space and time scales and were approached using modern methods of theoretical and computational physics, such as scaling analysis and diagrammatic techniques used before in critical phenomena theory. Special attention is paid to the asymptotics of concentration behavior (concentration tails). This issue is exceptionally important for the reliability assessments of radioactive waste disposal because, depending on the structure of the tails, concentrations at large distances from the source can differ by many orders of magnitude. In the first paper of this special section, Bolshov et al. (2008b) present an overview of field and laboratory observations that demonstrate nonclassical flow and transport behavior in geologic media. It is recognized that natural fracture networks as a rule have fractal geometry and can be classified as percolation systems. This is one of the main factors giving rise to anomalous transport in geologic media. Another important factor is the presence of contaminant traps provided by

  20. Assessment of the validity of sonography, computed tomography and angiography in diagnosis of the adrenals

    International Nuclear Information System (INIS)

    Georgi, M.; Hofbauer, J.; Weiss, H.; Keller, W.; Wunschik, F.; Mittelstaedt, G. von; Linder, M.; Heidelberg Univ., Mannheim; Heidelberg Univ., Mannheim

    1984-01-01

    Between 1979 and 1982, 47 patients with pathological process of the adrenals were surgically treated after preceding thorough non-evasive angiographic diagnosis (6 phaeochromocytomas, 20 adrenal adenomas, 8 adrenal hyperplasias, 5 adrenal carcinomas, 5 adrenal cysts and 3 adrenal metastases). The most consistently accurate results were obtained via adrenal phlebography with an accuracy of 94%. This was followed by non-evasive computed tomography (87%). Compared with the data given in literature, hormone determination in the adrenal venous blood was less favourable (79%). Selective adrenal arteriography, which was used less often, yielded correct results in 83% of the cases, whereas the number of accurate diagnoses achieved via sonography was lowest with 54%. (orig.) [de

  1. Computed tomography in the diagnosis of adrenal disease

    International Nuclear Information System (INIS)

    Hirosawa, Kunihiro

    1980-01-01

    From June 1977 through June 1980, sixty-one patients who were suspected to have adrenal diseases were examined with a CT scanner at Tokyo Women's Medical College. They consist of twenty five primary hyperaldosteronism, eight Cushing's syndrome, twenty pheochromocytoma and eight other adrenal masses. Ten patients were unexpectedly found to have adrenal lesion or mass simulating an adrenal tumor on CT performed for other reasons. CT findings were reviewed and correlated with surgical findings, postmortem studies and with results of other diagnostic modalities. 1. Primary hyperaldosteronism. Fifteen of twenty-five patients underwent surgery. Thirteen were pathologically proved to have aldosteronoma and two hyperplasia. Ten of thirteen patients with aldosteronoma were correctly diagnosed by CT scan. 2. Cushing's syndrome. Unilateral adenoma was correctly diagnosed preoperatively by CT scan on two surgically proved cases. CT showed marked enlargement of the adrenal gland with multiple nodules measuring less than 2 cm in diameter in the patient with nodular hyperplasia. Four patients were found to have normal-appearing adrenals with CT scan. 3. Pheochromocytoma. Three adrenal and one juxta-adrenal pheochromocytomas were detected by CT scan. Pheochromocytoma was considered as very unlikely on the basis of CT scan as well as further clinical investigation in sixteen patients. The value of CT scan for localization of extraadrenal pheochromocytoma remains established. 4. Miscellaneous adrenal disease and extra-adrenal masses simulating adrenal lesions. Two primary carcinoma, two bilateral metastasis, two adrenal neuroblastoma and a cyst were detected by CT scan. In cases with a huge mass, however, the origin and histologic diagnosis could not always be determined by CT scan. (author)

  2. Validity of computerized tomography in adrenal diseases

    International Nuclear Information System (INIS)

    Galanski, M.; Cramer, B.M.; Vetter, H.

    1981-01-01

    For diagnosis of adenomas in primary aldosteronism CT proved to be helpful. Although microadenomas have to be ruled out by selective venous sampling from both adrenal glands adenomas down to 8 mm in diameter can be visualized. Scintigraphy does not always permit reliable differentiation of unilateral adenoma from bilateral hyperplasia. - In endogenous Cushing's syndrome adrenal carcinomas are underlying etiologically in about 10% of the cases. Since carcinomas are detected easily by CT it should be performed in the first place. Whereas adenomas and hyperplasia are easily diagnosed by scintigraphy carcinomas may escape scintigraphy. - Pheochromocytomas, if situated in the vicinity of the kidneys, can be localiced safely by CT. Contrary to adenomas, carcinomas and pheochromocytomas may be detected by CT without difficulty because of their size. (orig.) [de

  3. Analysis of adrenocortical hyperplasia by computed tomography in patients with Cushing's disease, idiopathic hyperaldosteronism and adrenogenital syndrome

    International Nuclear Information System (INIS)

    Komiya, Ichiro; Ohara, Noriko; Nagasawa, Yoshitaka; Asawa, Takayuki; Hashizume, Kiyoshi; Takasu, Nobuyuki; Yamada, Takashi.

    1994-01-01

    We assessed the usefulness and reliability of computed tomography (CT scan) in evaluating adrenal hyperplasia in 38 patients, including 14 with Cushing's disease, 17 with idiopathic hyperaldosteronism (IHA), and 7 with the adrenogenital syndrome (AGS). Eighty-two normal subjects were also examined. We analyzed the shape of the adrenal gland and quantitated its thickness, width and length. Visual inspection revealed V-shaped right adrenal glands in 100% of patients with Cushing's disease, 94% of patients with IHA, 100% of patients with AGS and in 41% of the normal subjects. Triangular left adrenal glands were observed in 100% of patients with Cushing's disease, 82% of patients with IHA, 67% of patients with AGS and in 12% of the normal subjects. Quantitative analysis showed that the right adrenal gland was significantly thicker and longer in patients with Cushing's disease, IHA and in those with AGS than in normal subjects. The right adrenal gland was significantly wider in the patients with Cushing's disease and AGS than in control subjects. The left adrenal gland was significantly wider and longer in patients with Cushing's disease and AGS than in the normal controls. Analysis of individual data indicated that the upper limit of normal for thickness of the right adrenal was 7 mm. Therefore, adrenal hyperplasia was strongly suggested when the right adrenal gland was more than 7 mm thick. Our findings suggest that the CT scan is useful and reliable in diagnosing adrenal hyperplasia. (author)

  4. Nonlinearity and nonclassicality in a nanomechanical resonator

    Energy Technology Data Exchange (ETDEWEB)

    Teklu, Berihu [Clermont Universite, Blaise Pascal University, CNRS, PHOTON-N2, Institut Pascal, Aubiere Cedex (France); Universita degli Studi di Milano, Dipartimento di Fisica, Milano (Italy); Ferraro, Alessandro; Paternostro, Mauro [Queen' s University, Centre for Theoretical Atomic, Molecular, and Optical Physics, School of Mathematics and Physics, Belfast (United Kingdom); Paris, Matteo G.A. [Universita degli Studi di Milano, Dipartimento di Fisica, Milano (Italy)

    2015-12-15

    We address quantitatively the relationship between the nonlinearity of a mechanical resonator and the nonclassicality of its ground state. In particular, we analyze the nonclassical properties of the nonlinear Duffing oscillator (being driven or not) as a paradigmatic example of a nonlinear nanomechanical resonator. We first discuss how to quantify the nonlinearity of this system and then show that the nonclassicality of the ground state, as measured by the volume occupied by the negative part of the Wigner function, monotonically increases with the nonlinearity in all the working regimes addressed in our study. Our results show quantitatively that nonlinearity is a resource to create nonclassical states in mechanical systems. (orig.)

  5. Nonclassical effects in plasmonics: an energy perspective to quantify nonclassical effects

    DEFF Research Database (Denmark)

    Yan, Wei; Mortensen, N. Asger

    2016-01-01

    Plasmons are commonly interpreted with classical electrodynamics, while nonclassical effects may influence the dynamics of plasmon resonances as the plasmon confinement approaches the few-nanometer scale. However, an unambiguous approach to quantify the degree of nonclassical dynamics remains. We...... propose a nonclassical-impact parameter (NCI) to characterize the degree of nonclassical effects from an energy perspective, i.e., which fraction of the total electromagnetic energy is attributed to classical electrodynamic terms and which fraction is correspondingly to be assigned to nonclassical degrees...

  6. Adrenal scintigraphy with Scintadren

    International Nuclear Information System (INIS)

    Dabasi, G.; Irto, I.; Hernady, T.; Balint, I.

    1983-01-01

    68 patients with various adrenal disorders have been examined using Scintadren /TRC Amersham, England/. The parameters of adrenal imaging under Dexamethason suppression and after its discontinuance were established

  7. 131I-19-iodocholesterol adrenal scanning in Cushing's syndrome

    International Nuclear Information System (INIS)

    Charbonnel, B.; Chatal, J.F.; Chupin, M.; Guillon, J.

    1976-01-01

    7 patients were investigated: 2 bilateral hyperplasia due to pituitary ACTH excess showed bilateral adrenal activity. 1 post-surgical remnant with recurrent Cushing's syndrome was detected. 1 adenoma showed unilateral intense activity and absent activity in the controlateral gland, even after ACTH treatment. 2 carcinomas were weakly imaged. In 1 case, hepatic metastasis showed 131 I-19-iodocholesterol uptake. Adrenal imaging with 131 I-19-iodocholesterol is not a good procedure for assessing hormonal function. It is a valuable and safe tool in the localization and diagnosis of adrenal lesions causing Cushing's syndrome, perhaps better than radiologic procedures. Radiation dosimetry is acceptable [fr

  8. Adrenal Gland Disorders: Condition Information

    Science.gov (United States)

    ... About Share Facebook Twitter Pinterest Email Print About Adrenal Gland Disorders The adrenal glands, located on the top of ... as estrogen and testosterone. What are adrenal gland disorders? Adrenal gland disorders occur when the adrenal glands do not ...

  9. Computed tomography of the adrenal glands

    International Nuclear Information System (INIS)

    Buck, J.; Reiser, U.; Heuck, F.

    1982-01-01

    Whole body CT opens a third dimension - in addition with the advantage of being a non-invasive method with relatively negligible risk. Both, the normal CT findings of the adrenal gland and the normal variants in shape and position are described. With help of morphometry and image processing measurements of the size of the adrenal gland of 20 healthy patients were made and are listed; not only the respective normal variants but also hypo-hyperplasia are pointed out. Some examples are suitable for the illustration of pathologic conditions, such as inflammation and benign and malignant primary and secondary neoplasis. Finally, the value of adrenal gland CT is discussed with reference to the other radiologic methods. (orig.)

  10. Diagnostic evaluation of the adrenal scanning using 131I-adosterol

    International Nuclear Information System (INIS)

    Sugawara, Seiya; Nakamura, Mamoru; Sawai, Yoshikazu; Fukuchi, Soitsu.

    1978-01-01

    We have performed adrenal scanning in the 30 patients with suspected adrenal disorders eight days after the intravenous administration of about 500 μCi of 131 I-adosterol (NCL-6- 131 I), using 5 inch crystal rectilinear scintiscanner. Successful image of the adrenals was obtained in all the patients. In the 30 patients, 27 were proved to have adrenal disorders by surgical and hormonal findings. In 13 patients with primary aldosteronism, the side of adrenal adenoma was diagnosed correctly in all the cases by adrenal scanning. We could detected a small aldosterone-producing adenoma which measured 11 x 8 x 6 mm in size. In two patients with idiopathic hyperaldosteronism, asymmetrical radio-uptake between the two adrenals was seen on the standard scanning, and it was difficult to differentiate between tumor or hyperplasia. Dexamethazone-modified suppression scanning was very effective in lateralizing adenomas in the patients with primary aldosteronism. Two patients with Cushing's syndrome due to adrenal hyperplasia showed prominent and almost equal radioactivity of both the adrenal glands. Adrenal adenomas in 8 patients with Cushing's syndrome were definitely visualized on adrenal scanning, with no uptake in the contralateral sides. In one patient with Cushing's syndrome due to an adrenal carcinoma, adrenal scanning showed significant activity in the area of the carcinoma, and no uptake on the opposite side. In one patient with adrenogenital syndrome due to a virilizing adenoma with focal malignancy, adrenal scanning showed high radioactivity in the region of the tumor, and moderate activity on the opposite side. Also in the case of adrenal carcinoma, we appreciated diagnostic value of the adrenal scanning utilizing 131 I-adosterol. (author)

  11. Hormonal and metabolic evaluation of adrenal incidentalomas.

    Science.gov (United States)

    Wagnerova, H; Dudasova, D; Lazurova, I

    2009-01-01

    The biochemical and hormonal data in patients with adrenal incidentalomas were evaluated to compare the differences between adrenal adenomas and other benign lesions and to find the relationship between metabolic parameters and adrenal hormones. Ninety two patients (29men, age 20-90 years) with incidentally discovered unilateral or bilateral adrenal masses detected on CT were included in this study for the reasons others than adrenal pathology. Glycemia, cholesterolemia, triglyceridemia, hormonal evaluation including plasma ACTH, plasma aldosterone, plasma renin acitivity, overnight dexametasone test, ACTH test, free plasma metanephrines, urinary catecholamines were determined. In the group of patients with adrenal masses the prevalence of arterial hypertension was three fold higher, the prevalence of DM was approximately five fold higher and the prevalence of the overweight and obesity two fold higher than is reported in the general population. The most frequent adrenal masses were nonfunctional masses, the occurence of functional lesions was as follows: steroid enzymopathies (an exaggerated response of 17-OHP indicating a possible 21-hydroxylase deficiency), subclinical Cushing syndrome, primary aldosteronism and pheochromocytoma (5%, 2%, 2% and 1% respectively). There were no significant differences in evaluated data between patients with adenomas and hyperplasia and also no significant difference in evaluated data between lesions smaller than 3 cm and lesions greater than 3 cm. We did not find any correlations between plasma cortisol and lipid values. In this study we confirmed a higher prevalence of symptoms characteristic for different metabolic syndromes in these patients with adrenal incidentalomas, which indicate systematic screening for the metabolic syndrome including evaluation of the insuline resistance in this patients.

  12. Benign prostate hyperplasia (BPH) - resources

    Science.gov (United States)

    Resources - benign prostatic hyperplasia (BPH); Prostate enlargement resources; BPH resources ... The following organizations provide information on benign prostatic hyperplasia ( prostate enlargement ... Urology Care Foundation -- www. ...

  13. Biosynthesis of Various Steroids in vitro by Isolated Adrenal Cells in Primary Aldosteronism, Cushing's Syndrome, and Adrenogenital Syndrome due to Adrenocortical Adenoma

    OpenAIRE

    MIZUNO, SHIGERU; FUNAHASHI, HIROOMI

    1981-01-01

    To a further understanding of the role of steroid hormones in adrenal disorders, we have prepared free cell system of adrenal cells, using adrenal tissues that had been removed by operation from (i) cases of Cushing's syndrome due to adrenocortical adenoma or adrenocortical hyperplasia, (ii) a case of primary aldosteronism, and (iii) a patient with virilizing adrenal tumor. Twelve important steroid hormones were measured, such as pregnenolone, cortisol and aldosterone, which were produced by ...

  14. Embryology of the adrenal glands and its relevance to diagnostic imaging

    Energy Technology Data Exchange (ETDEWEB)

    Barwick, T.D. [Department of Diagnostic Radiology, St Bartholomew' s Hospital, London (United Kingdom)]. E-mail: tara.barwick@btinternet.com; Malhotra, A. [Department of Diagnostic Radiology, St Bartholomew' s Hospital, London (United Kingdom); Webb, J.A.W. [Department of Diagnostic Radiology, St Bartholomew' s Hospital, London (United Kingdom); Savage, M.O. [Department of Paediatric Endocrinology, St Bartholomew' s Hospital, London (United Kingdom); Reznek, R.H. [Department of Diagnostic Radiology, St Bartholomew' s Hospital, London (United Kingdom)

    2005-09-01

    An understanding of the embryology of the adrenal glands is necessary to appreciate the location of adrenal ectopic, or rest, tissue which can occur anywhere along the course of gonadal descent. This tissue usually has no clinical significance, but may become hyperplastic in patients with primary or secondary adrenal pathology. In congenital adrenal hyperplasia, hyperplastic rest tissue may present as a soft-tissue mass, particularly in the gonads and retroperitoneum, and may be mistaken for tumour. The adrenal in the neonate is proportionately much larger than in the adult; in renal ectopy or agenesis the ipsilateral adrenal is normally sited and may be mistaken for a kidney because of its size. This review article illustrates the embryology of the adrenal with particular emphasis on the relevance of embryology to pathology.

  15. Exponential attractors for a nonclassical diffusion equation

    Directory of Open Access Journals (Sweden)

    Qiaozhen Ma

    2009-01-01

    Full Text Available In this article, we prove the existence of exponential attractors for a nonclassical diffusion equation in ${H^{2}(Omega}cap{H}^{1}_{0}(Omega$ when the space dimension is less than 4.

  16. Fisher information, nonclassicality and quantum revivals

    Energy Technology Data Exchange (ETDEWEB)

    Romera, Elvira [Instituto Carlos I de Física Teórica y Computacional, Universidad de Granada, Fuentenueva s/n, 18071 Granada (Spain); Departamento de Física Atómica, Molecular y Nuclear, Universidad de Granada, Fuentenueva s/n, 18071 Granada (Spain); Santos, Francisco de los, E-mail: dlsantos@onsager.ugr.es [Instituto Carlos I de Física Teórica y Computacional, Universidad de Granada, Fuentenueva s/n, 18071 Granada (Spain); Departamento de Electromagnetismo y Física de la Materia, Universidad de Granada, Fuentenueva s/n, 18071 Granada (Spain)

    2013-11-08

    Wave packet revivals and fractional revivals are studied by means of a measure of nonclassicality based on the Fisher information. In particular, we show that the spreading and the regeneration of initially Gaussian wave packets in a quantum bouncer and in the infinite square-well correspond, respectively, to high and low nonclassicality values. This result is in accordance with the physical expectations that at a quantum revival wave packets almost recover their initial shape and the classical motion revives temporarily afterward.

  17. Angiolymphoid Hyperplasia With Eosinophilia

    Directory of Open Access Journals (Sweden)

    Rath Namita

    2002-01-01

    Full Text Available Angiolymphoid hyperplasia with eosinophilia is a disease of the occident. It is mainly seen in middle aged women. It presents as multiple small pink or purple popular or nodular eruptions, in the head and neck area. Lesions of angiolymphoid hyperplasia with eosinophilia are often confused with lesions of Kimura’s disease, which is more common in young males. We report a case of angiolymphoid hyperplasia with eosinophilia in a 34 year old female. The patient is responding to monthly intralesional triamcinolone acetate along with cryotherapy with liquid nitrogen. This case is being reported due to its rarity in Indian patients.

  18. Adrenal Gland Tumors: Statistics

    Science.gov (United States)

    ... Gland Tumor: Statistics Request Permissions Adrenal Gland Tumor: Statistics Approved by the Cancer.Net Editorial Board , 03/ ... primary adrenal gland tumor is very uncommon. Exact statistics are not available for this type of tumor ...

  19. Adrenal Gland Cancer

    Science.gov (United States)

    ... either benign or malignant. Benign tumors aren't cancer. Malignant ones are. Most adrenal gland tumors are ... and may not require treatment. Malignant adrenal gland cancers are uncommon. Types of tumors include Adrenocortical carcinoma - ...

  20. Adrenal Gland Disorders

    Science.gov (United States)

    ... stress and has many other important functions. With adrenal gland disorders, your glands make too much or not enough ... born unable to make enough cortisol. Causes of adrenal gland disorders include Genetic mutations Tumors including pheochromocytomas Infections A ...

  1. The prevalence of incidentally detected adrenal enlargement on CT

    International Nuclear Information System (INIS)

    Tang, Y.Z.; Bharwani, N.; Micco, M.; Akker, S.; Rockall, A.G.; Sahdev, A.

    2014-01-01

    Aim: To assess the prevalence and the department's detection rate of adrenocortical hyperplasia. Materials and methods: All computed tomography (CT) examinations of the adrenal glands between February and April 2011 were reviewed. The study excluded patients with known underlying cancer, abdominal trauma, or endocrine disease. The adrenal gland was deemed enlarged if its body was greater than 10 mm diameter, or a limb greater than 5 mm. Results: There were a total of 564 eligible CT studies during this period. A total of 64 cases of incidental adrenal enlargement were found giving a prevalence of 11.3%. Only nine cases were reported in the contemporaneous CT report. Conclusion: The results of the present study show that incidental adrenal enlargement has a significant prevalence. It is often dismissed during reporting, and awareness needs to be raised in the radiological community. Equally, the clinical and biochemical significance needs to be assessed with endocrine correlation

  2. Effect of the dose of oral Hydrocortisone on growth rate during long-term treatment of children with salt losing congenital adrenal hyperplasia Efecto de la dosis oral de hidrocortisona sobre la velocidad de crecimiento en niños con la forma perdedora de sodio de la hiperplasia suprarrenal congénita

    Directory of Open Access Journals (Sweden)

    M. Ciaccio

    2002-12-01

    Full Text Available The effect of the dose of oral hydrocortisone on stature growth rate of patients with the salt losing form of congenital adrenal hyperplasia and adequate electrolyte balance was here assessed. Thirty patients (21 girls and 9 boys were followed longitudinally for 0.52 to 8.64 years, between chronological ages 0.35 and 8.64 years. Nine consecutive periods (Ps of follow up were defined in order to compare two auxological parameters, height (H at the end of a follow up P and DH standard deviation score (SDS. According to growth rate during a particular P, two types of Ps were defined: Ps with DH SDS > -0.5 (Group 1, satisfactory growth rate and Ps with DH SDS = or 18 mg/m²/day and grew poorly, but they were able to recover, at least temporarily, when the dose was adjusted during the following years.El propósito del estudio fue evaluar el efecto de la dosis dehidrocortisona sobre la velocidad de crecimiento en pacientes con la forma perdedora de sodio de la hiperplasia suprarrenal congénita, en adecuado balance hidroelectrolítico. Se siguieron en forma longitudinal 30 pacientes (21 niñas y 9 niños durante 0.52 a 8.64 años, con edades cronológicas entre 0.35 y 8.64 años. Se definieron 9 períodos (P consecutivos de seguimiento para comparar: el score de desviación estándar (SDS para talla (T al final del P y el DSDS T (diferencia en el SDS de talla. Se definieron dos tipos de Ps: P con DSDS T > -0.5 (Grupo 1, velocidad de crecimiento satisfactoria y P con DSDS = o 18 mg/m²/día y crecieron poco. Sin embargo, la talla se recuperó cuando la dosis fue ajustada en años posteriores.

  3. Impaired cognitive function in women with congenital adrenal hyperplasia

    DEFF Research Database (Denmark)

    Johannsen, Trine H.; Ripa, Caroline P.L.; Reinisch, June M.

    2006-01-01

    of the Wechsler Adult Intelligence Scale was used, i.e. full-scale intelligence quotient (IQ; five of 11 subtests), which included three of six verbal IQ subtests and two of five performance IQ subtests. RESULTS: A significantly lower IQ was found in CAH patients compared with controls with respect to mean full...

  4. Congenital adrenal hyperplasia due to 21-hydroxylase deficiency in ...

    African Journals Online (AJOL)

    was measured by Cisbio radioimmunoassay (Cisbio, France), and serum aldosterone and 17-OHP were measured by .... Compared with SV patients, notably more CSW patients (62.1%, n=18) had severe acute malnutrition. SV patients were more likely to be obese (n=5, 45.5%) or overweight (n=6, 54.5%) at presentation ...

  5. Feminizing genitoplasty in congenital adrenal hyperplasia: the value ...

    African Journals Online (AJOL)

    genitogram. Clitoroplasty, vaginoplasty, and labioplasty were performed. The common sinus was managed by urogenital mobilization. Postoperative cosmetic ... disorder with an incidence of 1/10 000–1/15 000 live births [1]. It results from inherited defects in one of the five enzymatic steps required for the biosynthesis of.

  6. Feminizing genitoplasty in congenital adrenal hyperplasia: the value ...

    African Journals Online (AJOL)

    Clitoroplasty, vaginoplasty, and labioplasty were performed. The common sinus was managed by urogenital mobilization. Postoperative cosmetic outcome was evaluated according to the criteria described by Creighton and colleagues. Follow-up included clinical assessment of the urethral and vaginal openings and clinical ...

  7. 131I-cholesterol (19-C) in the diagnosis of adrenal disorders

    International Nuclear Information System (INIS)

    Mueller, C.; Glanzmann, C.; Luetolf, U.M.; Renk, I.W.; Horst, W.

    1976-01-01

    Results are reported of quantitative adrenal scintigraphy with 131 I-cholesterol in 8 patients with normal adrenal function before and after ACTH-stimulation and cortisol-suppression respectively, in one case with an adrenal metastasis from a pulmonary cancer, in two cases with Cushing's disease and in 7 cases with Conn's disease. In normal cases and in patients with M. Cushing a diagnosis of adrenal cortical function is possible with this method but can usually be replaced by more specific biochemical studies of serum and urine. The scintigraphic method is in addition of value in localization studies and can in unequivocal cases replace the selective adrenal phlebography. In patients with M. Conn increased uptake values (bilateral adrenal cortex hyperplasia) as well as reduced or non-detectable uptakes were observed, one of these cases suffering from a cortex carcinoma with M. Conn. (orig.) [de

  8. Selenium-75-cholesterol imaging and computed tomography of the adrenal glands in differentiating the cause of Cushing's syndrome

    International Nuclear Information System (INIS)

    Miller, J.L.; Smith, J.A.; Mervis, B.; Roman, T.

    1983-01-01

    Measurement of 75 Se-cholesterol (Scintadren) uptake and computed tomography (CT) of the adrenal glands were compared as a means of differentiating the cause of Cushing's syndrome in 11 patients over a 2-year period. Quantitative Scintadren imaging differentiated adrenocorticotrophic hormone (ACTH)-dependent disease from local adrenocortical lesions as the cause of Cushing's syndrome in all the patients studied. CT of the adrenal glands rapidly and accurately detected the adrenal mass lesions in 2 cases and was effective in documenting bilateral hyperplasia due to ectopic ACTH-dependent disease. However, in entopic ACTH (pituitary)-dependent disease the adrenal glands were of normal thickness in all but 2 patients, who had bilateral hyperplasia. Scintadren imaging and CT are useful non-invasive procedures for differentiating local adrenal disease from ACTH-dependent disease as the cause of Cushing's syndrome and should be the initial investigations once a firm clinical and biochemical diagnosis of Cushing's syndrome has been made

  9. Surfactant enhanced non-classical extraction

    International Nuclear Information System (INIS)

    Szymanowski, J.

    1999-01-01

    Surfactant enhanced non-classical extractions are presented and discussed. They include micellar enhanced ultrafiltration and cloud point extraction. The ideas of the processes are given and the main features are presented. They are compared to the classical solvent extraction. The fundamental of micellar solutions and their solubilization abilities are also discussed. (author)

  10. Nonclassical Orthogonal Polynomials and Corresponding Quadratures

    CERN Document Server

    Fukuda, H; Alt, E O; Matveenko, A V

    2004-01-01

    We construct nonclassical orthogonal polynomials and calculate abscissas and weights of Gaussian quadrature for arbitrary weight and interval. The program is written by Mathematica and it works if moment integrals are given analytically. The result is a FORTRAN subroutine ready to utilize the quadrature.

  11. Nonclassical rotational inertia in helium crystals.

    Science.gov (United States)

    Clark, A C; West, J T; Chan, M H W

    2007-09-28

    It has been proposed that the observed nonclassical rotational inertia (NCRI) in solid helium results from the superflow of thin liquid films along interconnected grain boundaries within the sample. We have observed NCRI in large (4)He crystals grown at constant temperature and pressure, demonstrating that the superfluid grain boundary model cannot explain the phenomenon.

  12. A rare adrenal incidentaloma: adrenal schwannoma.

    Science.gov (United States)

    Adas, Mine; Ozulker, Filiz; Adas, Gokhan; Koc, Bora; Ozulker, Tamer; Sahin, Ilknur Mansuroglu

    2013-01-01

    Adrenal schwannoma is an extremely uncommon cause of incidentaloma. It originates from neural sheath Schwann cells of the adrenal gland. We report the case of a left adrenal schwannoma incidentally discovered in a 32-year-old woman during examination of bloated feeling and stomach ache. The patient was incidentally found to have a left adrenal mass of 9 cm on abdominal ultrasonography. Computed tomography (CT) of the abdomen and [(18)F] fluorodeoxyglucose positron emission tomography (PET) were also performed. Metabolic evaluation was unremarkable. Due to the large size of the tumor, left adrenalectomy was performed. The postoperative course was uneventful. Histological examination established the diagnosis of schwannoma. This diagnosis was supported by immunohistochemistry of S-100 and vimentin positivity. In conclusion, adrenal schwannoma is an extremely rare entity and can grow considerably in size. The present case report emphasizes that clinicians should be aware of the possibility of retroperitoneal schwannoma. Total excision of benign schwannoma is associated with a favorable outcome. To our knowledge, there are case reports of schwannoma with CT and magnetic resonance imaging findings in the literature, although this is the first schwannoma case with PET-CT imaging.

  13. Acute adrenal crisis

    Science.gov (United States)

    ... due to, for example, Addison disease or other adrenal gland disease, and surgery The pituitary is injured and cannot ... A.M. Editorial team. Addison Disease Read more Adrenal Gland Disorders Read more NIH MedlinePlus Magazine Read more A. ...

  14. Chapter 13. Adrenal glands

    International Nuclear Information System (INIS)

    Roux, H.; Paulin, R.

    1975-01-01

    The condition of isotopic methods to the functional and morphological exploration of the adrenal glands is shown, with emphasis on the fact that althought the cortico-adrenal responds to these methods the same does not apply to the medullo-adrenal, which expresses its morphological changes by producing deformations on the cortical image. Funtional tests, mainly directed at the cortico-adrenal, are described first: study of exchangeable sodium and potassium; determination of the plasma concentration and metabolic clearance of some steroid hormones (cortisol, corticosterone, aldosterone); evaluation of the renin activity. These tests are based on competitive analysis and radioimmunological methods. Morphological tests are examined next. Adrenal scintigraphy uses a simple technique (intraveinous administration of 131 I 19-iodocholesterol with no special preliminary preparation) which gives good images and is only limited now by the need to avoid over exposure of the gonads to ionising radiations [fr

  15. Nonclassical energy transfer in photosynthetic FMO complex

    Directory of Open Access Journals (Sweden)

    Abramavicius Vytautas

    2013-03-01

    Full Text Available Excitation energy transfer in a photosynthetic FMO complex has been simulated using the stochastic Schrödinger equation. Fluctuating chromophore transition energies are simulated from the quantum correlation function which allows to properly include the finite temperature. The resulting excitation dynamics shows fast thermalization of chromophore occupations into proper thermal equilibrium. The relaxation process is characterized by entropy dynamics, which shows nonclassical behavior.

  16. Primary adrenal sarcomatoid carcinoma

    Directory of Open Access Journals (Sweden)

    Aftab S. Shaikh

    2014-03-01

    Full Text Available Adrenal sarcomatoid carcinomas are extremely rare tumors presenting with extensive locoregional spread at the time of diagnosis. Patients succumb to metastases within a couple of months. As a result, very few cases are reported in the literature until now. We present a case of a 62-year old female with non-functional sarcomatoid carcinoma of the right adrenal gland. There was no radiological evidence of locoregional metastases. Patient underwent right adrenalectomy. Follow up after 3 months showed para-aortic lymphadenopathy and similar left adrenal mass on computed tomography. Patient refused further treatment and succumbed to the disease. A brief case report with review of literature is presented.

  17. Angular-momentum nonclassicality by breaking classical bounds on statistics

    International Nuclear Information System (INIS)

    Luis, Alfredo; Rivas, Angel

    2011-01-01

    We derive simple practical procedures revealing the quantum behavior of angular momentum variables by the violation of classical upper bounds on the statistics. Data analysis is minimum and definite conclusions are obtained without evaluation of moments, or any other more sophisticated procedures. These nonclassical tests are very general and independent of other typical quantum signatures of nonclassical behavior such as sub-Poissonian statistics, squeezing, or oscillatory statistics, being insensitive to the nonclassical behavior displayed by other variables.

  18. Treatment of Nonclassic 11-Hydroxylase Deficiency with Ashwagandha Root

    Directory of Open Access Journals (Sweden)

    Daniel Powell

    2017-01-01

    Full Text Available An elderly woman presented with acne and male pattern alopecia, which upon diagnostic evaluation was found to be due to nonclassic 11-hydroxylase deficiency. We previously reported that Ashwagandha root ameliorates nonclassic 3-β-ol dehydrogenase and aldosterone synthase deficiencies. This is the first report of its use being associated with amelioration of nonclassic 11-hydroxylase deficiency, where its apparent effects appear to be dose-related.

  19. Non-classical continuum mechanics a dictionary

    CERN Document Server

    Maugin, Gérard A

    2017-01-01

    This dictionary offers clear and reliable explanations of over 100 keywords covering the entire field of non-classical continuum mechanics and generalized mechanics, including the theory of elasticity, heat conduction, thermodynamic and electromagnetic continua, as well as applied mathematics. Every entry includes the historical background and the underlying theory, basic equations and typical applications. The reference list for each entry provides a link to the original articles and the most important in-depth theoretical works. Last but not least, every entry is followed by a cross-reference to other related subject entries in the dictionary.

  20. Emission tomography for adrenal imaging

    International Nuclear Information System (INIS)

    Britton, K.E.; Shapiro, B.; Hawkins, L.A.

    1980-01-01

    Single photon emission tomography (SPET) of the adrenals was compared to convential gamma camera images. Depths of 19 adrenals were assessed by both the lateral skin-upper kidney pole method and by SPET. Eleven patients with adrenal disorders were also studied. An advantage of using SPET was that the analogue transverse section image showed improvement over the conventional posterior view because the liver activity was well separated from the adrenal. Furthermore, non-adrenal tissue background was virtually eliminated and adrenal depth determination facilitated. (U.K.)

  1. Classical and nonclassical randomness in quantum measurements

    International Nuclear Information System (INIS)

    Farenick, Douglas; Plosker, Sarah; Smith, Jerrod

    2011-01-01

    The space POVM H (X) of positive operator-valued probability measures on the Borel sets of a compact (or even locally compact) Hausdorff space X with values in B(H), the algebra of linear operators acting on a d-dimensional Hilbert space H, is studied from the perspectives of classical and nonclassical convexity through a transform Γ that associates any positive operator-valued measure ν with a certain completely positive linear map Γ(ν) of the homogeneous C*-algebra C(X) x B(H) into B(H). This association is achieved by using an operator-valued integral in which nonclassical random variables (that is, operator-valued functions) are integrated with respect to positive operator-valued measures and which has the feature that the integral of a random quantum effect is itself a quantum effect. A left inverse Ω for Γ yields an integral representation, along the lines of the classical Riesz representation theorem for linear functionals on C(X), of certain (but not all) unital completely positive linear maps φ:C(X) x B(H)→B(H). The extremal and C*-extremal points of POVM H (X) are determined.

  2. Nonclassical nucleation pathways in protein crystallization

    Science.gov (United States)

    Zhang, Fajun

    2017-11-01

    Classical nucleation theory (CNT), which was established about 90 years ago, has been very successful in many research fields, and continues to be the most commonly used theory in describing the nucleation process. For a fluid-to-solid phase transition, CNT states that the solute molecules in a supersaturated solution reversibly form small clusters. Once the cluster size reaches a critical value, it becomes thermodynamically stable and favored for further growth. One of the most important assumptions of CNT is that the nucleation process is described by one reaction coordinate and all order parameters proceed simultaneously. Recent studies in experiments, computer simulations and theory have revealed nonclassical features in the early stage of nucleation. In particular, the decoupling of order parameters involved during a fluid-to-solid transition leads to the so-called two-step nucleation mechanism, in which a metastable intermediate phase (MIP) exists between the initial supersaturated solution and the final crystals. Depending on the exact free energy landscapes, the MIPs can be a high density liquid phase, mesoscopic clusters, or a pre-ordered state. In this review, we focus on the studies of nonclassical pathways in protein crystallization and discuss the applications of the various scenarios of two-step nucleation theory. In particular, we focus on protein solutions in the presence of multivalent salts, which serve as a model protein system to study the nucleation pathways. We wish to point out the unique features of proteins as model systems for further studies.

  3. Congenital hemifacial hyperplasia

    Directory of Open Access Journals (Sweden)

    S A Deshingkar

    2011-01-01

    Full Text Available Congenital hemifacial hyperplasia (CHH is a rare congenital malformation characterized by marked unilateral overdevelopment of hard and soft tissues of the face. Asymmetry in CHH is usually evident at birth and accentuated with age, especially at puberty. The affected side grows at a rate proportional to the nonaffected side so that the disproportion is maintained thr oughout the life. Multisystem involvement has resulted in etiological heterogeneity including heredity, chromosomal abnormalities, atypical forms of twinning, altered intrauterine environment, and endocrine dysfunctions; however, no single theory explains the etiology adequately. Deformities of all tissues of face, including teeth and their related tissues in the jaw, are key findings for correct diagnosis of CHH. Here an attempt has been made to present a case of CHH with its archetypal features and to supplement existing clinical knowledge.

  4. [Surgery of adrenal tumors].

    Science.gov (United States)

    Bondarenko, V O; Ermolov, A S; Kovalenko, T I; Kondratiev, A V

    2004-01-01

    From 1983 to 2003 examination and surgical treatment were performed in 463 patients with different adrenal tumors. Hormone-active tumors were revealed in 249 of them, non-active - in 214. Combination of CT or MRT with study of adrenal hormones is the basis of the diagnosis. In different cases multispiral computed tomography, angiography, selected taking of blood from inferior caval vein, US- or CT-guided biopsy were used. Open surgery through thoracofrenolumbotomy was performed in 392 patients, videolaparoscopic surgery - in 71. Expediency of laparoscopic surgery in line with open surgery is demonstrated.

  5. Imaging of adrenal disorders

    International Nuclear Information System (INIS)

    Fukuchi, Soitsu

    1982-01-01

    Adrenal scintillation scanning, CT and ultrasonography are compared with the conventional imaging methods. The accuracy of retroperitoneal pneumography and adrenal venography are not high, and they detected only large tumors such as Cushing's syndrome and pheochromocytoma. Scintillation scanning is highly effective for the diagnoses of primary aldosteronism and Cushing's syndrome. However, this technique does not visualize pheochromocytoma or hypopituitarism. CT is noninvasive and of high diagnostic value. It is impossible to diagnose tumors by ultrasonography unless the size is more than 3 cm. (Chiba, N.)

  6. GATA transcription factors in testicular adrenal rest tumours.

    Science.gov (United States)

    Engels, Manon; Span, Paul N; Mitchell, Rod T; Heuvel, Joop J T M; Marijnissen-van Zanten, Monica A; van Herwaarden, Antonius E; Hulsbergen-van de Kaa, Christina A; Oosterwijk, Egbert; Stikkelbroeck, Nike M; Smith, Lee B; Sweep, Fred C G J; Claahsen-van der Grinten, Hedi L

    2017-11-01

    Testicular adrenal rest tumours (TARTs) are benign adrenal-like testicular tumours that frequently occur in male patients with congenital adrenal hyperplasia. Recently, GATA transcription factors have been linked to the development of TARTs in mice. The aim of our study was to determine GATA expression in human TARTs and other steroidogenic tissues. We determined GATA expression in TARTs ( n  = 16), Leydig cell tumours (LCTs; n  = 7), adrenal (foetal ( n  = 6) + adult ( n  = 10)) and testis (foetal ( n  = 13) + adult ( n  = 8)). We found testis-like GATA4 , and adrenal-like GATA3 and GATA6 gene expressions by qPCR in human TARTs, indicating mixed testicular and adrenal characteristics of TARTs. Currently, no marker is available to discriminate TARTs from LCTs, leading to misdiagnosis and incorrect treatment. GATA3 and GATA6 mRNAs exhibited excellent discriminative power (area under the curve of 0.908 and 0.816, respectively), while immunohistochemistry did not. GATA genes contain several CREB-binding sites and incubation with 0.1 mM dibutyryl cAMP for 4 h stimulated GATA3 , GATA4 and GATA6 expressions in a human foetal testis cell line (hs181.tes). Incubation of adrenocortical cells (H295RA) with ACTH, however, did not induce GATA expression in vitro Although ACTH did not dysregulate GATA expression in the only human ACTH-sensitive in vitro model available, our results do suggest that aberrant expression of GATA transcription factors in human TARTs might be involved in TART formation. © 2017 The authors.

  7. Managing Adrenal Insufficiency

    Science.gov (United States)

    ... temporary AI include the following: • Transsphenoidal surgery for Cushing’s disease that removes a tumor from the pituitary gland • ... adrenal glands that regulates carbohydrate and protein metabolism Cushing’s disease A pituitary tumor that makes too much ACTH ...

  8. Surgery for adrenal tumors

    International Nuclear Information System (INIS)

    Salamah, S.M.

    2002-01-01

    Objective: To analyze the presentation, localization, pathology, surgical management and outcome of surgery for adrenal gland tumors. Design: Prospective clinico epidemiological study. Place and Duration of Study: The study was conducted at the Department of General Surgery, University Unit, Riyadh medical Complex Kingdom of Saudi Rabia from June, 1991 to may, 2001. Subjects and Methods: A total of 21 cases with adrenal tumors were studied for demographic data, clinical presentation, diagnostic workup, localization, surgical management, pathology and outcome. The outcome of these patients was followed prospectively. Results: The study included 12 female and 9 male patients. The mean age at surgery was 36.7 years. Hypertension (69.%) was the commonest presentation in hypersecretory functional tumors. The localization accuracy for ultrasonography, computerized tomography, MRI and MIBG scan was 95.2%, 98.3% 87.8% and 83.6% respectively. Pheochromocytoma was the most common adrenal pathology observed in 14 (66.6%) cases. The overall morbidity was 19% with no hospital mortality. Complete follow-up of available 19 patients (90.5 %) revealed no tumor recurrence and persistent hypertension in 14.3% cases. Conclusion: surgery on adrenal glands is safe in experienced hands and is recommended in institutes with all backup facilities. (author)

  9. Nonclassical Kinetics of Clonal yet Heterogeneous Enzymes.

    Science.gov (United States)

    Park, Seong Jun; Song, Sanggeun; Jeong, In-Chun; Koh, Hye Ran; Kim, Ji-Hyun; Sung, Jaeyoung

    2017-07-06

    Enzyme-to-enzyme variation in the catalytic rate is ubiquitous among single enzymes created from the same genetic information, which persists over the lifetimes of living cells. Despite advances in single-enzyme technologies, the lack of an enzyme reaction model accounting for the heterogeneous activity of single enzymes has hindered a quantitative understanding of the nonclassical stochastic outcome of single enzyme systems. Here we present a new statistical kinetics and exactly solvable models for clonal yet heterogeneous enzymes with possibly nonergodic state dynamics and state-dependent reactivity, which enable a quantitative understanding of modern single-enzyme experimental results for the mean and fluctuation in the number of product molecules created by single enzymes. We also propose a new experimental measure of the heterogeneity and nonergodicity for a system of enzymes.

  10. Testing dimension and nonclassicality in communication networks

    Science.gov (United States)

    Bowles, Joseph; Brunner, Nicolas; Pawłowski, Marcin

    2015-08-01

    We consider networks featuring preparation, transformation, and measurement devices, in which devices exchange communication via mediating physical systems. We investigate the problem of testing the dimension of the mediating systems in the device-independent scenario, that is, based on observable data alone. A general framework for tackling this problem is presented, considering both classical and quantum systems. These methods can then also be used to certify the nonclassicality of the mediating systems, given an upper bound on their dimension. Several case studies are reported, which illustrate the relevance of the framework. These examples also show that, for fixed dimension, quantum systems largely outperform classical ones. Moreover, the use of a transformation device considerably improves noise tolerance when compared to simple prepare-and-measure networks. These results suggest that the classical simulation of quantum systems becomes costly in terms of dimension, even for simple networks.

  11. Strategic management of adrenal tumors.

    Science.gov (United States)

    Schmidt, N

    1994-02-01

    Adrenal tumors--large and small, symptomatic and asymptomatic, benign and malignant--are being diagnosed with increasing frequency. Patients presenting with endocrine syndromes symptomatic of adrenal tumors require meticulous, considerate history taking, thorough physical examination, and careful planning for medical management and/or surgical removal. Adrenal tumors discovered in the absence of endocrine syndromes, and which are greater than 5 cm in diameter, require careful biochemical and imaging investigation before removal, since their rate of malignancy increases with size. Adrenal masses less than 4 cm in diameter present in the absence of endocrine syndromes can, after full endocrine and imaging evaluation, be carefully observed. Even with optimal surgical/medical management, malignant disease of the adrenal glands presents a poor outcome. This paper reviews the broad management of adrenal tumors, including a personal experience with a challenging case of adrenal adenocarcinoma.

  12. Nonclassical states of the Jaynes - Cummings model and its excitation

    International Nuclear Information System (INIS)

    Verlan, E.M.; Razumova, M.A.

    2002-01-01

    The nonclassical squeezed states of the Jaynes -Cummings (JC) model are built,and the problem of their excitation in parametric processes is considered.The statistical properties of a field oscillator are analyzed in these states

  13. Nonclassical pseudospectral method for the solution of brachistochrone problem

    International Nuclear Information System (INIS)

    Alipanah, A.; Razzaghi, M.; Dehghan, M.

    2007-01-01

    In this paper, nonclassical pseudospectral method is proposed for solving the classic brachistochrone problem. The brachistochrone problem is first formulated as a nonlinear optimal control problem. Properties of nonclassical pseudospectral method are presented, these properties are then utilized to reduce the computation of brachistochrone problem to the solution of algebraic equations. Using this method, the solution to the brachistochrone problem is compared with those in the literature

  14. On the origin of nonclassicality in single systems

    International Nuclear Information System (INIS)

    Aravinda, S; Srikanth, R; Pathak, Anirban

    2017-01-01

    In the framework of certain general probability theories of single systems, we identify various nonclassical features such as incompatibility, multiple pure-state decomposability, measurement disturbance, no-cloning and the impossibility of certain universal operations, with the non-simpliciality of the state space. This is shown to naturally suggest an underlying simplex as an ontological model. Contextuality turns out to be an independent nonclassical feature, arising from the intransitivity of compatibility. (paper)

  15. Classical and nonclassical symmetries analysis for initial value problems

    International Nuclear Information System (INIS)

    Zhang Zhiyong; Chen Yufu

    2010-01-01

    Classical and nonclassical symmetries are considered to reduce evolution equations with initial conditions in two independent variables. First of all, we rearrange the classical infinitesimal operators such that they leave the initial value problems invariant. Secondly, we give a sufficient condition for the nonclassical symmetry reductions of initial value problems. The generalized Kuramoto-Sivashinsky equation with dispersive effects is considered to examine the algorithms.

  16. Nonclassical pseudospectral method for the solution of brachistochrone problem

    Energy Technology Data Exchange (ETDEWEB)

    Alipanah, A. [Department of Applied Mathematics, Amirkabir University of Technology, Tehran (Iran, Islamic Republic of); Razzaghi, M. [Department of Mathematics and Statistics, Mississippi State University, Mississippi State, MS 39762 (United States) and Department of Applied Mathematics, Amirkabir University of Technology, Tehran (Iran, Islamic Republic of)]. E-mail: razzaghi@math.msstate.edu; Dehghan, M. [Department of Applied Mathematics, Amirkabir University of Technology, Tehran (Iran, Islamic Republic of)

    2007-12-15

    In this paper, nonclassical pseudospectral method is proposed for solving the classic brachistochrone problem. The brachistochrone problem is first formulated as a nonlinear optimal control problem. Properties of nonclassical pseudospectral method are presented, these properties are then utilized to reduce the computation of brachistochrone problem to the solution of algebraic equations. Using this method, the solution to the brachistochrone problem is compared with those in the literature.

  17. Characterization of Classical and Nonclassical Fabry Disease: A Multicenter Study.

    Science.gov (United States)

    Arends, Maarten; Wanner, Christoph; Hughes, Derralynn; Mehta, Atul; Oder, Daniel; Watkinson, Oliver T; Elliott, Perry M; Linthorst, Gabor E; Wijburg, Frits A; Biegstraaten, Marieke; Hollak, Carla E

    2017-05-01

    Fabry disease leads to renal, cardiac, and cerebrovascular manifestations. Phenotypic differences between classically and nonclassically affected patients are evident, but there are few data on the natural course of classical and nonclassical disease in men and women. To describe the natural course of Fabry disease stratified by sex and phenotype, we retrospectively assessed event-free survival from birth to the first clinical visit (before enzyme replacement therapy) in 499 adult patients (mean age 43 years old; 41% men; 57% with the classical phenotype) from three international centers of excellence. We classified patients by phenotype on the basis of characteristic symptoms and enzyme activity. Men and women with classical Fabry disease had higher event rate than did those with nonclassical disease (hazard ratio for men, 5.63, 95% confidence interval, 3.17 to 10.00; P Fabry disease had lower eGFR, higher left ventricular mass, and higher plasma globotriaosylsphingosine concentrations than men with nonclassical Fabry disease or women with either phenotype ( P Fabry disease had a history of more events than men with nonclassical disease or women with either phenotype; women with classical Fabry disease were more likely to develop complications than women with nonclassical disease. These data may support the development of new guidelines for the monitoring and treatment of Fabry disease and studies on the effects of intervention in subgroups of patients. Copyright © 2017 by the American Society of Nephrology.

  18. In silico analyses reveal nuclear asymmetry of spongiocytes and compact cells of adrenocorticotrophic hormone-independent macronodular adrenocortical hyperplasia.

    Science.gov (United States)

    Zhang, Qian; Dou, Jingtao; Gu, Weijun; Yang, Guoqing; Mu, Yiming; Lu, Juming

    2014-05-01

    Little information is available about the risk of progression of seemingly benign adrenocortical hyperplasias to carcinomatous conditions. Using in silico approaches of digitally archived tissue sections, the nuclear morphometric parameters were compared to assess nuclear asymmetry as an index for nuclear atypia. Four groups of nuclei were used for the current study: spongiocytes and compact cells obtained from adrenocorticotropic hormone (ACTH)-independent macronodular hyperplasia, which were hypothesized to be high risk for nuclear asymmetry, and primary pigmented nodular adrenocortical disease and micronodular adrenocortical hyperplasia samples were used as internal controls. Analyses reveal high nuclear irregularity index of spongiocytes and shape factor abnormalities of both spongiocytes and compact cells of ACTH-independent macronodular adrenal hyperplasia compared with the other 2 groups (high F values and very low P values after analyses of variances), thus confirming the hypothesis that ACTH-independent macronodular adrenal hyperplasia present with subtle morphometric features of nuclear atypia. This probably puts this class of adrenocortical tumors at risk of dysplastic progression, and more studies are needed to test the hypothesis.

  19. Overtraining, Exercise, and Adrenal Insufficiency

    OpenAIRE

    Brooks, KA; Carter, JG

    2013-01-01

    Running, or any aerobic training in moderation, has a positive effect on health. There is a point of diminishing returns, where chronic stress from overtraining, which is common in runners, may be linked to problems in the adrenal gland. Overtraining Syndrome (OS) has been linked with adrenal insufficiency. There is a direct link between stress and the adrenal glands, and the physical stress of overtraining may cause the hormones produced in these glands to become depleted. Overtraining Syndr...

  20. Isolated adrenal paracoccidioidomycosis: Case report

    International Nuclear Information System (INIS)

    Uribe Castro, Jorge Ricardo; Quintana, Humberto; Puentes, Alix Sofia and others

    2011-01-01

    Even though paracoccidioidomycosis has a relatively high prevalence in Latin America in a systemic form, isolated cases, especially compromising the adrenal glands, are uncommon, with only two reported cases. In this article, we report the case of a 55 year-old male with clinical manifestations of adrenal insufficiency. The only imaging finding was the presence of bilateral adrenal masses. The biopsy showed Paracoccidioides brasiliensis infection.

  1. How Is Adrenal Surgery Performed?

    Science.gov (United States)

    ... Frequently Asked Questions Office visit worksheet ALSO SEE: NEUROENDOCRINE SYSTEM PARATHYROID GLANDS THYROID GLANDS How is adrenal surgery performed? This document is available in ... Parathyroid Glands | Thyroid Gland

  2. Adenomatoid hyperplasia of lower lip

    Directory of Open Access Journals (Sweden)

    Gaganjot Kaur Sharma

    2011-01-01

    Full Text Available Adenomatoid hyperplasia (AH is an uncommon, non-neoplastic swelling on the palate caused due to hyperplasia of the mucinous acini. The lesion clinically presents as a sessile tumor-like nodule resembling pleomorphic adenoma. Histopathologic findings include lobules of enlarged mucinous acini which are filled with secretory granules. The nuclei are squeezed to the basal portions, associated with focal inflammation and ductal dilatation, and a history of trauma is often elicited. Here, we report a rare case of AH of the lower lip in a 20-year-old male patient, which mimics a mucous retention cyst or mucocele.

  3. Thymic hyperplasia in Graves′ disease

    Directory of Open Access Journals (Sweden)

    Narendra Kotwal

    2013-01-01

    Full Text Available Graves′ disease is an autoimmune thyroid condition characterized by the production of autoantibodies against the thyrotropin receptor. It is known to be associated with autoimmune conditions such as myasthenia gravis, Addison′s disease, type 1 diabetes mellitus, and vitiligo. We present a case of rare autoimmune association of Graves′ disease with thymic hyperplasia which regressed after treatment with antithyroid drugs. Exact pathophysiology of thymic hyperplasia in Graves′ is not well understood; it is likely to be the result of rather than the cause of Graves′ disease.

  4. Adrenal Histoplasmosis in Immunocompetent Patients Presenting as Adrenal Insufficiency.

    Science.gov (United States)

    Gajendra, Smeeta; Sharma, Rashi; Goel, Shalini; Goel, Ruchika; Lipi, Lipika; Sarin, Hemanti; Guleria, Mridula; Sachdev, Ritesh

    2016-01-01

    Histoplasmosis is an infectious disease caused by the dimorphic fungus Histoplasma capsulatum, endemic in central and eastern states of United States, South America and Africa. India is considered to be non-endemic area for histoplasmosis. Disseminated histoplasmosis may affect almost all systems. Disseminated histoplasmosis with asymptomatic adrenal involvement has been described in immunocompromised patients; whereas isolated adrenal involvement with adrenal insufficiency as the presenting manifestation of the disease is rare. Twelve patients from a non-endemic area with adrenal histoplasmosis, who were immunocompetent and diagnosed as adrenal histoplasmosis by cytology/histopathology between January 2012 to December 2014 were studied. 18F-FDG PET/CT (fluorodeoxyglucose positron emission tomography/computed tomography) was used to assess the extent of involvement. There were a total of 12 immunocompetent males (mean age: 56.9 years). Ten patients had bilateral adrenal involvement and two had a unilateral left adrenal mass. All the patients had histopathologically/cytologically proven adrenal histoplasmosis. Two patients had simultaneous histoplasmosis of other sites, one in the epiglottis and the other in the alveolus. 18F-FDG PET/CT was performed in 10 patients showing high FDG uptake in the adrenals. All these patients received Amphotericin B and/or Itraconazole treatment that led to symptomatic improvement. A diagnosis of invasive fungal infection requires a high index of suspicion, especially in immunocompetent patients who present with nonspecific symptoms, clinical signs, laboratory and radiological features that can resemble adrenal neoplasms. Clinical specimens must be sent for cytopathology/histopathology together with fungal culture for a definite diagnosis and appropriate management.

  5. Ovarian Leydig Cell Hyperplasia: An Unusual Case of Virilization in a Postmenopausal Woman

    Directory of Open Access Journals (Sweden)

    Jaya M. Mehta

    2014-01-01

    Full Text Available Objective. To report an unusual case of ovarian Leydig cell hyperplasia resulting in virilization in a postmenopausal woman. Methods. Patient’s medical history and pertinent literature were reviewed. Results. A 64-year-old woman presented with virilization with worsening hirsutism, deepening of her voice, male musculature, and male pattern alopecia. Her pertinent past medical history included type 1 diabetes, hyperlipidemia, and hypertension. Her pertinent past surgical history included hysterectomy due to fibroids. On further work-up, her serum total testosterone was 506 ng/dL (nl range: 2–45 and free testosterone was 40 pg/mL (nl range: 0.1–6.4. After ruling out adrenal causes, the patient underwent an empiric bilateral oophorectomy that showed Leydig cell hyperplasia on pathology. Six weeks postoperatively, serum testosterone was undetectable with significant clinical improvement. Conclusion. Postmenopausal hyperandrogenism can be the result of numerous etiologies ranging from normal physiologic changes to ovarian or rarely adrenal tumors. Our patient was found to have Leydig cell hyperplasia of her ovaries, a rarely reported cause of virilization.

  6. Atom ionization in a nonclassical intense electromagnetic field

    International Nuclear Information System (INIS)

    Popov, A.M.; Tikhonova, O.V.

    2002-01-01

    The atoms ionization process in the intense nonclassical electromagnetic field is considered. It is shown that depending on the field quantum state the probability of ionization may essentially change even by one and the same average quantum number in the radiation mode, whereby the difference in the ionization rates is especially significant in the case, when the ionization process is of a multiphoton character. It is demonstrates in particular, that the nonclassical field may be considerably more intensive from the viewpoint of the atoms ionization, than the classical field with the same intensity. The peculiarities of the decay, related to the atomic system state in the strong nonclassical field beyond the perturbation theory frames are studied [ru

  7. A single-centre experience of the implementation of adrenal vein sampling procedure: the impact on the diagnostic work-up in primary aldosteronism

    NARCIS (Netherlands)

    Kadziela, J.; Prejbisz, A.; Michalowska, I.; Kolodziejczyk-Kruk, S.; Schultze Kool, L.J.; Kabat, M.; Janaszek-Sitkowska, H.; Toutounchi, S.; Galazka, Z.; Ambroziak, U.; Bednarczuk, T.; Ptasinska-Wnuk, D.; Hoffmann, M.; Januszewicz, M.; Januszewicz, A.; Witkowski, A.

    2017-01-01

    BACKGROUND: Primary aldosteronism is one of the most common causes of secondary hypertension. Adrenal vein sampling (AVS) remains a "gold standard" procedure in differentiation between unilateral (adenoma) and bilateral (hyperplasia) disease. AIM: The aim of this study was to present our

  8. The role of computed tomography in assessment of endocrine adrenal disorders in adult patients. Report of fifty-six cases

    International Nuclear Information System (INIS)

    Laissy, J.P.; Genevois, A.; Assailly, C.; Janvresse, A.; Louvel, J.P.; Benozio, M.; Fournier, L.; Denizet, D.; Clement, J.F.

    1987-01-01

    A prospective CT study has been performed in 56 patients in order to define its usefulness in the diagnosis of adrenal masses accompanying an endocrine syndrome. CT data have been compared with clinical, biological and surgical data. CT scan accuracy is variable with different endocrine disorders, better in pheochromocytoma than in Cushing syndrome. In this syndrome, size of the mass and density after contrast media injection may differentiate adenoma from hyperplasia in difficult cases. In hyperaldosteronism, significant differences in densities contribute to the diagnosis between adenoma and hyperplasia [fr

  9. Long-term Results after CT-Guided Percutaneous Ethanol Ablation for the Treatment of Hyperfunctioning Adrenal Disorders

    Directory of Open Access Journals (Sweden)

    Nathan Elie Frenk

    Full Text Available OBJECTIVES: To evaluate the safety and long-term efficacy of computed tomography-guided percutaneous ethanol ablation for benign primary and secondary hyperfunctioning adrenal disorders. METHOD: We retrospectively evaluated the long-term results of nine patients treated with computed tomography-guided percutaneous ethanol ablation: eight subjects who presented with primary adrenal disorders, such as pheochromocytoma, primary macronodular adrenal hyperplasia and aldosterone-producing adenoma, and one subject with Cushing disease refractory to conventional treatment. Eleven sessions were performed for the nine patients. The patient data were reviewed for the clinical outcome and procedure-related complications over ten years. RESULTS: Patients with aldosterone-producing adenoma had clinical improvement: symptoms recurred in one case 96 months after ethanol ablation, and the other patient was still in remission 110 months later. All patients with pheochromocytoma had clinical improvement but were eventually submitted to surgery for complete remission. No significant clinical improvement was seen in patients with hypercortisolism due to primary macronodular adrenal hyperplasia or Cushing disease. Major complications were seen in five of the eleven procedures and included cardiovascular instability and myocardial infarction. Minor complications attributed to sedation were seen in two patients. CONCLUSION: Computed tomography-guided ethanol ablation does not appear to be suitable for the long-term treatment of hyperfunctioning adrenal disorders and is not without risks.

  10. Long-term results after CT-guided percutaneous ethanol ablation for the treatment of hyper functioning adrenal disorders

    International Nuclear Information System (INIS)

    Frenk, Nathan Elie; Sebastianes, Fernando; Lerario, Antonio Marcondes; Fragoso, Maria Candida Barisson Villares; Mendonca, Berenice Bilharinho

    2016-01-01

    Objectives: To evaluate the safety and long-term efficacy of computed tomography-guided percutaneous ethanol ablation for benign primary and secondary hyper functioning adrenal disorders. Method: We retrospectively evaluated the long-term results of nine patients treated with computed tomography guided percutaneous ethanol ablation: eight subjects who presented with primary adrenal disorders, such as pheochromocytoma, primary macro nodular adrenal hyperplasia and aldosterone-producing adenoma, and one subject with Cushing disease refractory to conventional treatment. Eleven sessions were performed for the nine patients. The patient data were reviewed for the clinical outcome and procedure-related complications over ten years. Results: Patients with aldosterone-producing adenoma had clinical improvement: symptoms recurred in one case 96 months after ethanol ablation, and the other patient was still in remission 110 months later. All patients with pheochromocytoma had clinical improvement but were eventually submitted to surgery for complete remission. No significant clinical improvement was seen in patients with hypercortisolism due to primary macro nodular adrenal hyperplasia or Cushing disease. Major complications were seen in five of the eleven procedures and included cardiovascular instability and myocardial infarction. Minor complications attributed to sedation were seen in two patients. Conclusion: Computed tomography-guided ethanol ablation does not appear to be suitable for the long-term treatment of hyper functioning adrenal disorders and is not without risks. (author)

  11. Long-term results after CT-guided percutaneous ethanol ablation for the treatment of hyper functioning adrenal disorders

    Energy Technology Data Exchange (ETDEWEB)

    Frenk, Nathan Elie; Sebastianes, Fernando; Lerario, Antonio Marcondes; Fragoso, Maria Candida Barisson Villares; Mendonca, Berenice Bilharinho [Universidade de Sao Paulo (USP), SP (Brazil). Faculdade de Medicina; Menezes, Marcos Roberto de, E-mail: menezesmr@gmail.com [Instituto do Cancer do Estado de Sao Paulo, SP (Brazil)

    2016-10-15

    Objectives: To evaluate the safety and long-term efficacy of computed tomography-guided percutaneous ethanol ablation for benign primary and secondary hyper functioning adrenal disorders. Method: We retrospectively evaluated the long-term results of nine patients treated with computed tomography guided percutaneous ethanol ablation: eight subjects who presented with primary adrenal disorders, such as pheochromocytoma, primary macro nodular adrenal hyperplasia and aldosterone-producing adenoma, and one subject with Cushing disease refractory to conventional treatment. Eleven sessions were performed for the nine patients. The patient data were reviewed for the clinical outcome and procedure-related complications over ten years. Results: Patients with aldosterone-producing adenoma had clinical improvement: symptoms recurred in one case 96 months after ethanol ablation, and the other patient was still in remission 110 months later. All patients with pheochromocytoma had clinical improvement but were eventually submitted to surgery for complete remission. No significant clinical improvement was seen in patients with hypercortisolism due to primary macro nodular adrenal hyperplasia or Cushing disease. Major complications were seen in five of the eleven procedures and included cardiovascular instability and myocardial infarction. Minor complications attributed to sedation were seen in two patients. Conclusion: Computed tomography-guided ethanol ablation does not appear to be suitable for the long-term treatment of hyper functioning adrenal disorders and is not without risks. (author)

  12. Diagnosis of adrenal tumors

    Energy Technology Data Exchange (ETDEWEB)

    Richter, E.I.; Loesch, H.

    1987-09-01

    Of 155 patients with adrenal disorders, 120 (77%) were correctly diagnosed as negative. There were no correlations between the results of computer tomography and phlebography or between computer tomography and laboratory tests. In 31 patients (20%) a correct diagnosis was obtained and these patients were sent to surgery. Four cases (3%) were shown to be false positive. In these cases (with one exception), both the computer tomography and phlebography results had been overinterpreted. Computer tomography was shown to be a method of high sensitivity and almost as great specificity. Tumors cannot be distinguished by phlebography; only pheochromocytoma shows a characteristic alteration of vessels in arteriograms. In general, an accurate diagnosis requires positive angiography (arterio- or phlebography) results and clear evidence of elevated hormone levels. Only then is surgery indicated.

  13. Diagnosis of adrenal tumors

    International Nuclear Information System (INIS)

    Richter, E.I.; Loesch, H.

    1987-01-01

    Of 155 patients with adrenal disorders, 120 (77%) were correctly diagnosed as negative. There were no correlations between the results of computer tomography and phlebography or between computer tomography and laboratory tests. In 31 patients (20%) a correct diagnosis was obtained and these patients were sent to surgery. Four cases (3%) were shown to be false positive. In these cases (with one exception), both the computer tomography and phlebography results had been overinterpreted. Computer tomography was shown to be a method of high sensitivity and almost as great specificity. Tumors cannot be distinguished by phlebography; only pheochromocytoma shows a characteristic alteration of vessels in arteriograms. In general, an accurate diagnosis requires positive angiography (arterio- or phlebography) results and clear evidence of elevated hormone levels. Only then is surgery indicated. (orig.) [de

  14. Angiolymphoid hyperplasia with follicular mucinosis

    Directory of Open Access Journals (Sweden)

    Joshi Rajiv

    2007-01-01

    Full Text Available Follicular mucinosis occurring along with angiolymphoid hyperplasia with eosinophils (ALHE has been described in a 54-year-old female. The patient presented with pruritic erythematous papules on the left frontoparietal scalp. Histopathological examination showed prominent blood vessels in the dermis lined by plump histiocytoid endothelial cells that were surrounded by a dense lymphoid infiltrate with numerous eosinophils; these findings are typical of angiolymphoid hyperplasia with eosinophils. Features of follicular mucinosis were observed in the same section with several hyperplastic follicular infundibula containing pools of mucin in the infundibular epithelium. The concurrent occurrence of these two distinct histopathological patterns in the same biopsy specimen has been described in only three cases to date.

  15. Condylar hyperplasia - A case report

    Directory of Open Access Journals (Sweden)

    Gowri P Bhandarkar

    2003-01-01

    Full Text Available Condylar hyperplasia is a rare malformation of non neoplastic origin, wherein the size and morphology of one of the two mandibular condyles is affected. It is reported as a self limiting process that is generally seen in patients between age group of 11 to 30 years. It appears as an acceleration of growth in young patients which arises at the same time of physiological condylar growth or as an unpredictable growth spurt in adult.

  16. Ectopic Thyroid in the Adrenal Presenting as an Adrenal Incidentaloma

    Directory of Open Access Journals (Sweden)

    Banu Aktaş Yılmaz

    2016-12-01

    Full Text Available Adrenal incidentalomas are clinical dilemmas for the clinicians. The work up, to differentiate between malignant and benign lesions, and hyperfunctioning and nonfunctioning lesions is mandatory before the consideration of surgical resection. Ectopic thyroid tissue located in the adrenal gland (ETTAG is a very rare condition. We report a case of ETTAG presenting with adrenal incidentaloma. A 57-year-old woman was admitted with incidental right adrenal mass. Hormone evaluation showed no hormonal activity. Magnetic resonance imaging revealed a 20x17 mm lobulated solid mass, which contained millimetric hypointense nodular areas consistent with calcifications. Loss of signal intensity on out-of-phase could not be evaluated because of the calcifications. Right adrenalectomy was performed to establish the histopathological diagnosis and to rule out malignancy. Histopathological diagnosis revealed ETTAG. Her medical history was positive for multinodular goiter and bilateral subtotal thyroidectomy 32 years ago. Thyroid ultrasonography showed residual thyroid tissue in both the right and left lobes, and colloid thyroid nodules. Fine needle aspiration biopsy from the nodules revealed benign nodules. The patient has been followed up for six years, and no change in thyroid nodule sizes and no evidence of metastatic foci have been detected. ETTAG would be considered in the differential diagnosis of adrenal mass showing no hormonal activity, especially when magnetic resonance images are not consistent with adrenal adenoma. Long follow-up duration of this case suggests that it was a benign condition.

  17. Investigation on reactivity of non-classical carbenes with sterically ...

    Indian Academy of Sciences (India)

    Home; Journals; Journal of Chemical Sciences; Volume 128; Issue 4. Investigation on reactivity of non-classical carbenes with sterically hindered Lewis acid, B(C6F5)3 under inert and open conditions. Arunabha Thakur Pavan K Vardhanapu Gonela Vijaykumar Sushil Ranjan Bhatta. Regular Articles Volume 128 Issue 4 ...

  18. Investigation on reactivity of non-classical carbenes with sterically ...

    Indian Academy of Sciences (India)

    (OH2). ] (1). As a part of our ongoing research interest in aNHC chemistry,24 the present study reports the reactivity of the strongly acidic, sterically encumbered borane. B(C6F5)3 towards non-classical carbenes as Lewis bases. Herein we report the isolation and characteri- zation of four new boron compounds resulted by ...

  19. Contour detection based on nonclassical receptive field inhibition

    NARCIS (Netherlands)

    Grigorescu, Cosmin; Petkov, Nicolai; Westenberg, Michel A.

    We propose a biologically motivated computational step, called nonclassical receptive field (non-CRF) inhibition, more generally surround inhibition or suppression, to improve contour detection in machine vision. Non-CRF inhibition is exhibited by 80% of the orientation-selective neurons in the

  20. Classical Sets and Non-Classical Sets: An Overview -38 ...

    Indian Academy of Sciences (India)

    Classical Sets and Non-Classical Sets: Sumita Basu is assistant professor of mathematics at Lady Braboume. College, Kolkata. Her research interests include artificial intelligence, automata theory, and mathematical logic. Keywords. Fuzzy sets, crisp sets, rough sets, law of excluded middle,. DeMorgan's laws. An Overview.

  1. Adrenal Insufficiency and Addison's Disease

    Science.gov (United States)

    ... blood sugar or hyperglycemia vitiligo, a loss of pigment on areas of the skin Infections Tuberculosis (TB), ... to ACTH, or a defect in adrenal hormone production medication-related causes, such as from anti-fungal ...

  2. Overtraining, Exercise, and Adrenal Insufficiency.

    Science.gov (United States)

    Brooks, Ka; Carter, Jg

    2013-02-16

    Running, or any aerobic training in moderation, has a positive effect on health. There is a point of diminishing returns, where chronic stress from overtraining, which is common in runners, may be linked to problems in the adrenal gland. Overtraining Syndrome (OS) has been linked with adrenal insufficiency. There is a direct link between stress and the adrenal glands, and the physical stress of overtraining may cause the hormones produced in these glands to become depleted. Overtraining Syndrome (OS) has been described as chronic fatigue, burnout and staleness, where an imbalance between training/competition, versus recovery occurs. Training alone is seldom the primary cause. In most cases, the total amount of stress on the athlete exceeds their capacity to cope. A triggering stressful event, along with the chronic overtraining, pushes the athlete to start developing symptoms of overtraining syndrome, which is far worse than classic overtraining. Overtraining can be a part of healthy training, if only done for a short period of time. Chronic overtraining is what leads to serious health problems, including adrenal insufficiency. Severe overtraining over an extended period can result in adrenal depletion. An Addison-Type overtraining syndrome, where the adrenal glands are no longer able to maintain proper hormone levels and athletic performance is severely compromised has been described by researchers. The purpose of this review is to describe the relationship between overtraining, chronic fatigue, and adrenal insufficiency and to address the overlap in these conditions, as well as examine critical research on the relationship between the dysfunction of the adrenal axis in over trained and stressed athletes.

  3. Lipomatosis: a diverse form of hemifacial hyperplasia

    International Nuclear Information System (INIS)

    Arora, Preeti Chawla; Umarji, Hemant R.; Arora, Aman; Ramaswami, Easwaran

    2012-01-01

    A case of hemifacial hyperplasia that presented with muscular, skeletal, and dental hyperplasia along with lipomatous infiltration was described. Advanced imaging was useful in identifying the lipomatous infiltration present in the lesion, which raises the possibility of lipomatosis having a diverse presentation in hemifacial hyperplasia. As there was a scarcity of related literature in the field of dentomaxillofacial radiology, this report would make us familiar with its computed tomographic and magnetic resonance image findings.

  4. Unilateral Condylar Hyperplasia of the Mandible

    Directory of Open Access Journals (Sweden)

    Malachovsky I

    2015-12-01

    Full Text Available Condylar hyperplasia (CH of the mandible is a rare pathology that occurs at the head of the condyle and can lead to facial asymmetry affecting occlusion and possible association with pain and dysfunction. Unilateral condylar hyperplasia is an uncommon condition of unknown aetiology, proper diagnosis of which has to be established, as the patients may look for surgical help. A rare case of unilateral condylar hyperplasia of the mandible is reported here.

  5. Lipomatosis: a diverse form of hemifacial hyperplasia

    Energy Technology Data Exchange (ETDEWEB)

    Arora, Preeti Chawla; Umarji, Hemant R. [Dept. of Oral Medicine, Diagnosis and Radiology, Government Dental College and Hospital, Mumbai (India); Arora, Aman [Dept. of Prosthodontics, Sri Guru Ram Das Institute of Dental Sciences and Research, Amritsar (India); Ramaswami, Easwaran [Oral Medicine, Diagnosis and Radiology, Dr. D.Y. Patil Dental College and Hospital, Navi Mumbai (India)

    2012-09-15

    A case of hemifacial hyperplasia that presented with muscular, skeletal, and dental hyperplasia along with lipomatous infiltration was described. Advanced imaging was useful in identifying the lipomatous infiltration present in the lesion, which raises the possibility of lipomatosis having a diverse presentation in hemifacial hyperplasia. As there was a scarcity of related literature in the field of dentomaxillofacial radiology, this report would make us familiar with its computed tomographic and magnetic resonance image findings.

  6. Quantitative selenium-75-cholesterol imaging and computed tomography of the adrenal glands in Conn's syndrome

    International Nuclear Information System (INIS)

    Miller, J.L.

    1982-01-01

    Six consecutive patients with biochemically proven primary aldosteronism (4 with unilateral aldosteronomas and 2 with bilateral hyperplasia) underwent imaging with 75 Se-selenomethyl-nor-cholesterol (Scintadren; Radiochemical Centre, Amersham, UK) and computed tomography (CT) of the adrenal glands to aid in lateralizing unilateral aldosterone-producing adenomas (APA) and to differentiate APA from idiopathic adrenal hyperplasia (IAH). Scintadren quantitative imaging alone was successful in lateralizing the lesion in all 4 cases of unilateral APA; mean uptake by the affected adrenal gland was 0,47% of the administered dose as against 0,23% in the normal gland (P smaller than 0,01). The mean uptake ratio for the adenomatous as against the normal gland was 2,03 (range 1,75-2,21), which was significantly greater than the uptake ratio of 0,82 in 4 normal individuals (P smaller than 0,01). CT lateralized all the APAs. In the 2 cases of IAH, Scintadren uptake was bilaterally increased in one case in which CT was normal, whereas in the other case Scintadren uptakes were normal while CT showed two abnormal glands. The overall diagnostic yield for Scintadren was 83%; the figure for CT was also 83%. When the results of Scintadren imaging and CT are pooled, the accuracy in lateralizing APAs and differentiating APA from IAH as a cause of Conn's syndrome is 100%

  7. Focal nodular hyperplasia in children

    International Nuclear Information System (INIS)

    Mancheva, Ts.; El Shemeri, S.; Balev, B.; Rasheva, N.; Georgieva, M.

    2016-01-01

    Focal nodular hyperplasia (FNH) is a rare benign tumor of the liver, which is extremely rare reported in children. We present a case of 6-month-old female. The infant was referred to the hospital for further investigation of incidentally established heterodense mass in the liver. Ultrasound and CT examinations were performed. They were combined with laboratory and viral serologic tests. The liver function tests were normal, except AFP. Viral serologic tests for hepatitis B and C were negative. Abdominal ultrasound revealed a heterodense mass, well-defined from the surrounding liver tissue. On Doppler examination, the blood supply is proven to be from a large artery. The findings on CT imaging include a solitary, homogeneous, and slightly hypoattenuating mass compared to the surrounding liver on unenhanced CT. After administration of i.v. contrast the lesion demonstrates rapid homogeneous contrast enhancement at the arterial phase (except for the central scar). On venous phase, the mass becomes isotense as compared to the surrounding liver. Although the diagnosis of FNH in children can be challenging for the radiologist, different imaging modalities like ultrasound, CT and MRI all offer specific advantages in the detection. Key words: FOCAL Nodular Hyperplasia. Imaging Diagnostic. Pediatric. Tumours In Children

  8. Hyperplasia

    Science.gov (United States)

    Kumar V, Abbas AK, Aster JC. Cellular responses to stress and toxic insults: adaptation, injury, and death. In: Kumar V, Abbas AK, Aster JC, eds. Robbins and Cotran Pathologic Basis of Disease . 9th ed. Philadelphia, PA: ...

  9. Laguerre polynomial excited coherent state: generation and nonclassical properties

    Science.gov (United States)

    Ye, Wei; Zhou, Weidong; Zhang, Haoliang; Liu, Cunjin; Huang, Jiehui; Hu, Liyun

    2017-11-01

    We propose a theoretical protocol to generate a kind of non-Gaussian state—a Laguerre polynomial excited coherent state (LPECS) by exploiting a two-mode squeezing transformation and a conditional measurement with a coherent state input. Then we investigate the nonclassical features of the LPECS according to the Glauber-Sudarshan P(α ) function, photon number distribution, Mandel’s Q parameter, second-order correlation function, and squeezing properties as well as negative Wigner distribution. Our results show that the generated output state presents obvious nonclassical properties which can be modulated by a coherent amplitude, a squeezing parameter and a conditional measurement. In particular, the squeezing and negative Wigner function are clear.

  10. Symplectic tomography of nonclassical states of trapped ion

    International Nuclear Information System (INIS)

    Man'ko, O.

    1996-03-01

    The marginal distribution for two types of nonclassical states of trapped ion - for squeezed and correlated states and for squeezed even and odd coherent states (squeezed Schroedinger cat states) is studied. The obtained marginal distribution for the two types of states is shown to satisfy classical dynamical equation equivalent to standard quantum evolution equation for density matrix (wave function) derived in symplectic tomography scheme. (author). 20 refs

  11. Atomism and the Reasoning by a Non-Classical Logic

    OpenAIRE

    Antonino Drago; Romina Oliva

    1999-01-01

    Often, in the original scientific writings, a double negated statement (DNS) is not equivalent to his corresponding positive one; that means the inferring law 'non non A -> A' does not apply. Recent studies recognized in the failure of this logical law the borderline between classical and non-classical logics. Original writings by classical chemists dealing with the problem of atomism are particularly characterized by the occurrences of DNSs. An historical case, Avogadro's contribution to ...

  12. Non-classical rotational inertia in the supersolid state

    OpenAIRE

    Koh, Shun-ichiro

    2004-01-01

    An abrupt drop in the moment of inertia recently found in solid helium 4 is explained in terms of dynamics of zero-point vacancies (ZPV). Mechanical decoupling of ZPV from the motion of the container due to Bose statistics is developed to a macroscopic phenomenon by repulsive interaction. It gives a negative answer to the question whether BEC is a necessary condition for non-classical rotational inertia in a bulk three-dimensional system.

  13. Bone Health in Adrenal Disorders

    Directory of Open Access Journals (Sweden)

    Beom-Jun Kim

    2018-03-01

    Full Text Available Secondary osteoporosis resulting from specific clinical disorders may be potentially reversible, and thus continuous efforts to find and adequately treat the secondary causes of skeletal fragility are critical to ameliorate fracture risk and to avoid unnecessary treatment with anti-osteoporotic drugs. Among the hyperfunctional adrenal masses, Cushing's syndrome, pheochromocytoma, and primary aldosteronism are receiving particularly great attention due to their high morbidity and mortality mainly by increasing cardiovascular risk. Interestingly, there is accumulating experimental and clinical evidence that adrenal hormones may have direct detrimental effects on bone metabolism as well. Thus, the present review discusses the possibility of adrenal disorders, especially focusing on pheochromocytoma and primary aldosteronism, as secondary causes of osteoporosis.

  14. Transcriptional regulation by nonclassical action of thyroid hormone

    Directory of Open Access Journals (Sweden)

    Moeller Lars C

    2011-08-01

    Full Text Available Abstract Thyroid hormone (TH is essential for normal development, growth and metabolism. Its effects were thought to be principally mediated through triiodothyronine (T3, acting as a ligand for the nuclear TH receptors (TRs α and β residing on thyroid hormone response elements (TREs in the promoter of TH target genes. In this classical model of TH action, T3 binding to TRs leads to recruitment of basal transcription factors and increased transcription of TH responsive genes. Recently, the concept of TH action on gene expression has become more diverse and now includes nonclassical actions of T3 and T4: T3 has been shown to activate PI3K via the TRs, which ultimately increases transcription of certain genes, e.g. HIF-1α. Additionally, both T3 and thyroxine (T4 can bind to a membrane integrin, αvβ3, which leads to activation of the PI3K and MAPK signal transduction pathways and finally also increases gene transcription, e.g. of the FGF2 gene. Therefore, these initially nongenomic, nonclassical actions seem to serve as additional interfaces for transcriptional regulation by TH. Aim of this perspective is to summarize the genes that are currently known to be induced by nonclassical TH action and the mechanisms involved.

  15. Improvement of semen quality in an infertile man with 21-hydroxylase deficiency, suppressed serum gonadotropins and testicular adrenal rest tumours

    DEFF Research Database (Denmark)

    Mouritsen, Annette; Juul, Anders; Jørgensen, Niels

    2010-01-01

    Here, we report improvement of semen quality in a 30-year-old man with congenital adrenal hyperplasia (CAH) because of 21-hydroxylase deficiency, bilateral testicular adrenal rest tumours (TART) and a 1.5-year infertility history. His adrenal substitution therapy was changed from hydrocortisone 10...... increased from 0.23 to 258.72 mio. The total number of morphologically normal spermatozoa increased from 0.01 to 19.6 mio. sp. The patient reported improved well being and did not develop signs of overtreatment. A non-optimal medical substitution therapy of this CAH patient is the most likely explanation...... for the presence of TART and disturbed reproductive hormones levels, leading to impaired semen quality. Optimizing the medical treatment may at least in some cases improve fecundity....

  16. Comparative assessment of adrenal function in Icenko-Cushing's disease according to the diagnostic pneumoretroperitoneum and morphological findings

    International Nuclear Information System (INIS)

    Tyurin, E.I.; Trofimov, V.M.

    1981-01-01

    A comparative analysis has been carried out, based on the adrenals X-ray trials, preceded by pneumoretroperitoneum, and surgical findings in 47 patients with Icenko-Cushing's disease and 20 to 50 years. Total bilateral adrenalectomy has been applied to surgical intervention. It has been established that the adrenals, either having normal morphology or showing signs of hyperplasia, have appeared unaltered in shape and size at tomograms. The X-ray picture peculiarities have been detected rather by the patients obesity rate, than the weight and histologic structure of the glands. Massive consolidation, found in most patients over the kidney at the plain X-ray film present no true enlargement of the adrenals, but should be considered as the reflection of the paranephral capsule, circumvening them [ru

  17. [Immunoendocrine associations in adrenal glands].

    Science.gov (United States)

    Sterzl, I; Hrdá, P

    2010-12-01

    Immune and endocrine systems are basic regulatory mechanisms of organism and, including the nervous system, maintain the organism's homeostasis. The main immune system representatives are mononuclear cells, T- and B-cells and their products, in the endocrine system the main representatives are cells of the glands with inner secretion and their products. One of the most important glands for maintaining homeostasis are adrenal glands. It has been proven that either cells of the immune system, either endocrine cells can, although in trace amounts, produce mutually mediators of both systems (hormones, cytokines). Disorders in one system can lead to pathological symptoms in the other system. Also here represent adrenals an important model.

  18. Bilateral primary adrenal lymphoma presenting with adrenal insufficiency

    DEFF Research Database (Denmark)

    Holm, Jakob; Breum, Leif; Stenfeldt, Katrine

    2012-01-01

    surgery was performed. A new computerized tomography scan showed rapid progression of disease with further enlargement of the adrenal masses and both pulmonary and hepatic metastasis. Needle biopsy was performed but the patient refused further treatment and died before a diagnosis was obtained...

  19. Neonatal adrenal hemorrhage presenting as acute scrotum

    African Journals Online (AJOL)

    Introduction. In newborns, adrenal hemorrhage is not an uncommon event. The large size of the adrenal cortex contributes to an increased vulnerability to trauma during a difficult delivery [1]. However, the neonatal adrenal hemorrhage may rarely present as inguinoscrotal swelling [2,3]. This condition can simulate torsion of ...

  20. Spontaneous Retroperitoneal Hemorrhage from Adrenal Artery Aneurysm

    International Nuclear Information System (INIS)

    Gonzalez Valverde, F.M.; Balsalobre, M.; Torregrosa, N.; Molto, M.; Gomez Ramos, M.J.; Vazquez Rojas, J.L.

    2007-01-01

    Spontaneous adrenal hemorrhage is a very rare but serious disorder of the adrenal gland that can require emergent treatment. We report on a 42-year-old man who underwent selective angiography for diagnosis and treatment of retroperitoneal hemorrhage from small adrenal artery aneurysm. This case gives further details about the value of transluminal artery embolization in the management of visceral aneurysm rupture

  1. [A case of multilocular cystic renal cell carcinoma treated by partial nephrectomy associated with adrenal tumor].

    Science.gov (United States)

    Fukuoka, H; Ishibashi, Y; Fujinami, K; Tsuchiya, F; Sakanishi, S

    1994-12-01

    A case of multilocular cystic renal cell carcinoma was reported. The patient was 69-year-old male who had been examined for postoperative study of gastric cancer by abdominal CT. The abdominal CT incidentally revealed right adrenal tumor which was non-functional and multilocular cysts in the lower pole of the right kidney. Selective renal arteriography showed a hypovascular mass with fine neovascularity. These two findings of CT and arteriography were though to represent a probable malignant tumor but renal function of the patient decreased moderately. Surgical exploration was done and right renal masses were thought to be seen benign multilocular cysts without capsule. Simple excision of the wall of cysts and right adrenalectomy were performed. Pathological examinations showed multilocular cystic renal cell carcinoma and benign adrenal hyperplasia. Additionally partial nephrectomy was done. Surgical margin of the kidney was tumor free and postoperative course was uneventful. Prognosis of multiocular cystic renal cell carcinoma is good, therefore conservative surgery is recommended.

  2. Anatomy of melancholia: focus on hypothalamic-pituitary-adrenal axis overactivity and the role of vasopressin.

    LENUS (Irish Health Repository)

    Dinan, Timothy G

    2012-02-03

    Overactivity of the hypothalamic-pituitary-adrenal (HPA) axis characterized by hypercortisolism, adrenal hyperplasia and abnormalities in negative feedback is the most consistently described biological abnormality in melancholic depression. Corticotropin-releasing hormone (CRH) and arginine vasopressin (AVP) are the main secretagogues of the HPA\\/stress system. Produced in the parvicellular division of the hypothalamic paraventricular nucleus the release of these peptides is influenced by inputs from monoaminergic neurones. In depression, anterior pituitary CRH1 receptors are down-regulated and response to CRH infusion is blunted. By contrast, vasopressin V3 receptors on the anterior pituitary show enhanced response to AVP stimulation and this enhancement plays a key role in maintaining HPA overactivity.

  3. Blood sampling from adrenal gland vein

    International Nuclear Information System (INIS)

    Sun Yong; Ni Caifang

    2009-01-01

    Adrenal gland vein sampling is an interventional method to get the blood samples from the adrenal gland vein. The blood is obtained via a catheter which is selectively inserted in the adrenal gland vein. This technique is mainly used to be diagnostic for primary hyperaldosteronism. A full knowledge of the anatomy and variations of the adrenal gland vein, serious preoperative preparation and skilled catheterization manipulation are necessary for obtaining sufficient blood sample and for reducing the occurrence of complications. Providing the physicians with definite diagnostic evidence and being technically feasible, adrenal gland vein sampling should become one of the routine examinations for clarifying the cause of primary hyperaldosteronism. (authors)

  4. Adrenal adrenoceptors in heart failure

    Directory of Open Access Journals (Sweden)

    Claudio ede Lucia

    2014-07-01

    Full Text Available Heart failure (HF is a chronic clinical syndrome characterized by the reduction in left ventricular (LV function and it represents one of the most important causes of morbidity and mortality worldwide. Despite considerable advances in pharmacological treatment, HF represents a severe clinical and social burden. Sympathetic outflow, characterized by increased circulating catecholamines (CAs biosynthesis and secretion, is peculiar in HF and sympatholytic treatments (as β-blockers are presently being investigated for the treatment of this disease. Adrenal gland secretes Epinephrine (80% and Norepinephrine (20% in response to acetylcholine stimulation of nicotinic cholinergic receptors on the chromaffin cell membranes. This process is regulated by adrenergic receptors (ARs: α2ARs inhibit CA release through coupling to inhibitory Gi-proteins, and βARs (mainly β2ARs stimulate CA release through coupling to stimulatory Gs-proteins. All ARs are G-protein-coupled receptors (GPCRs and GPCR kinases (GRKs regulate their signaling and function. Adrenal GRK2-mediated α2AR desensitization and downregulation are increased in HF and seem to be a fundamental regulator of CA secretion from the adrenal gland. Consequently, restoration of adrenal a2AR signaling through the inhibition of GRK2 is a fascinating sympatholytic therapeutic strategy for chronic HF. This strategy could have several significant advantages over existing HF pharmacotherapies (antiadrenergic, such as bAR-blockers minimizing side-effects on extra-cardiac tissues and reducing the chronic activation of the renin–angiotensin–aldosterone and endothelin systems.The role of adrenal ARs in regulation of sympathetic hyperactivity opens interesting perspectives in understanding pathophysiology of HF and identifying new potential therapeutic targets.

  5. Clinical and laboratory evaluation of adrenal dysfunction

    International Nuclear Information System (INIS)

    Ashkar, F.S.; Fishman, L.M.

    1983-01-01

    Because of their special physical and chemical properties, the adrenal secretory products were among the first hormonal substances to be measured by methods other than bioassay. Over the past several years, the development of sensitive and specific methods of hormone assay dependent on the use of radionuclides has revolutionized investigative and clinical endocrinology. While the capacity of defining most abnormalities of adrenal function antedates hormone measurement and adrenal imaging utilizing radioisotopes, the availability of such methods has greatly facilitated and made more precise the diagnostic approach to patients with suspected adrenal dysfunction. As an example of how clinical and laboratory considerations can be integrated into a rational approach to the diagnosis of adrenal disease, the problem of suspected adrenal hyperfunction is analyzed in light of current understanding of its pathophysiology. Reflection demonstrates that suspected primary aldosteronism and adrenal insufficiency are equally amenable to such an approach

  6. Adrenal failure due to bilateral adrenal metastasis of rectal cancer: A case report.

    Science.gov (United States)

    Imaoka, Yuki; Kuranishi, Fumito; Ogawa, Yoshiteru; Okuda, Hiroshi; Nakahara, Masahiro

    2017-01-01

    It is rare for a patient to present with adrenal insufficiency secondary to bilateral adrenal metastases from a malignant colorectal tumor. An 82-year-old Japanese man presented to our hospital with high fever and malaise. He was receiving oral chemotherapy for the treatment of rectal cancer with multiple metastases. Computed tomography showed new bilateral adrenal gland metastases. A rapid adrenocorticotropic hormone (ACTH) test showed adrenal insufficiency. Treatment with hydrocortisone provided immediate symptom improvement. Adrenal insufficiency secondary to bilateral adrenal metastases from rectal cancer is rare. A rapid ACTH test is useful to diagnose adrenal insufficiency. The incidence of adrenal insufficiency may be underestimated in patients with multiple metastasis. Appropriate therapy with adrenal corticosteroid hormone supplementation may lead to a significant improvement in the patient's symptoms and quality of life. Copyright © 2016 The Author(s). Published by Elsevier Ltd.. All rights reserved.

  7. Angiolymphoid Hyperplasia with Eosinophilia: A Case Report.

    Science.gov (United States)

    Esteves, Paola; Barbalho, Marcella; Lima, Tiago; Quintella, Leonardo; Niemeyer-Corbellini, João Paulo; Ramos-E-Silva, Marcia

    2015-01-01

    Angiolymphoid hyperplasia with eosinophilia is a rare disease considered as being a vascular malformation resulting from a subjacent arteriovenous shunt. It affects mostly the head of women between 20 and 40 years old and may present spontaneous involution.

  8. Thermotherapy and thermoablation for benign prostatic hyperplasia

    NARCIS (Netherlands)

    Gravas, Stavros; Laguna, Pilar; de la Rosette, Jean

    2003-01-01

    PURPOSE OF REVIEW: From all the available thermoablative methods for the treatment of symptomatic benign prostatic hyperplasia, transurethral microwave thermotherapy is considered as standard in minimally invasive management. The literature is enriched by several new studies on transurethral

  9. A prenatally detected adrenal cyst treated by adrenal-sparing ...

    African Journals Online (AJOL)

    Ahmet Dursun and Munevver Ho ¸sgo¨ r. A neonatal case of left adrenal cyst detected in utero and ... Correspondence to Munevver Ho ¸sgör, MD, PhD, Department II of Pediatric. Surgery, Dr Behcet Uz Children's Hospital, Koruturk Mh. ..... Radiology 1986; 161:631–633. 13 Erbil Y, Salmasliog˘lu A, Barbaros U, Bozbora A, ...

  10. Patrolling Mechanics of Non-Classical Monocytes in Vascular Inflammation.

    Science.gov (United States)

    Buscher, Konrad; Marcovecchio, Paola; Hedrick, Catherine C; Ley, Klaus

    2017-01-01

    Non-classical monocytes have emerged as the preeminent vascular housekeepers. Continuous intravascular screening is enabled by slow patrolling on the endothelium and allows a rapid response to local perturbations. Intravital imaging has been crucial to elucidate the molecular mechanisms and migratory phenotype of patrolling. In this review, we discuss technical requirements of intravital microscopy such as imaging modalities, labeling strategies, and data analysis. We further focus on patrolling kinetics and adhesion receptors in different organs and vascular beds including arteries during homeostasis and vascular inflammation and define pertinent questions in the field.

  11. Patrolling Mechanics of Non-Classical Monocytes in Vascular Inflammation

    Directory of Open Access Journals (Sweden)

    Konrad Buscher

    2017-12-01

    Full Text Available Non-classical monocytes have emerged as the preeminent vascular housekeepers. Continuous intravascular screening is enabled by slow patrolling on the endothelium and allows a rapid response to local perturbations. Intravital imaging has been crucial to elucidate the molecular mechanisms and migratory phenotype of patrolling. In this review, we discuss technical requirements of intravital microscopy such as imaging modalities, labeling strategies, and data analysis. We further focus on patrolling kinetics and adhesion receptors in different organs and vascular beds including arteries during homeostasis and vascular inflammation and define pertinent questions in the field.

  12. Nonclassical photon streams using rephased amplified spontaneous emission

    International Nuclear Information System (INIS)

    Ledingham, Patrick M.; Naylor, William R.; Longdell, Jevon J.; Beavan, Sarah E.; Sellars, Matthew J.

    2010-01-01

    We present a fully quantum mechanical treatment of optically rephased photon echoes. These echoes exhibit noise due to amplified spontaneous emission; however, this noise can be seen as a consequence of the entanglement between the atoms and the output light. With a rephasing pulse one can get an 'echo' of the amplified spontaneous emission, leading to light with nonclassical correlations at points separated in time, which is of interest in the context of building wide bandwith quantum repeaters. We also suggest a wideband version of DLCZ protocol based on the same ideas.

  13. Quantum nondemolition measurement with a nonclassical meter input and an electro-optic enhancement

    DEFF Research Database (Denmark)

    Andersen, Ulrik Lund; Buchler, B.C.; Bachor, H.A.

    2002-01-01

    Optical quantum nondemolition measurements are performed using a beamsplitter with a nonclassical meter input and a electro-optic feedforward loop. The nonclassical meter input is provided by a stable 4.5 dB amplitude squeezed source generated by an optical parametric amplifier. We show...

  14. Long-Term Gynecological Outcomes in Women with Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency

    DEFF Research Database (Denmark)

    Johannsen, T H; Ripa, Caroline P.L.; Carlsen, E

    2010-01-01

    Background. Our knowledge on long-term outcome in CAH remains incomplete. Methods. In a prospective study (33 CAH patients, 33 age-matched controls), reproductive outcomes, self-rating of genital appearance and function, and sexuality were correlated to degree of initial virilisation, genotype.......3-10.8] versus 2.8 [0.6-10.8], P = .09) than controls. Satisfaction with genital appearance was lower and negatively correlated to degree of initial virilisation (r(s) = ≤-0.39, P ≤ .05). More patients had never had intercourse (P = .001), and age at 1st intercourse was higher (18 yrs versus 16 yrs, P = .02...

  15. Gender Dysphoria in a 62-Year-Old Genetic Female With Congenital Adrenal Hyperplasia.

    Science.gov (United States)

    Silveira, Mariana Telles; Knobloch, Felícia; Silva Janovsky, Carolina C P; Kater, Claudio E

    2016-10-01

    We report a case of gender dysphoria (GD) in a 62-year-old genetic female patient, raising the pros and cons of performing corrective surgery later in life. This 46,XX DSD patient was registered and reared as a girl; CAH was diagnosed late in childhood. Poor adherence to treatment and lack of proper psychological management contributed to evident GD. Living for years as a male, the patient applied for a legitimate male identification document in his late 50s; thereafter, he requested a sex-reassignment surgery "to disguise his female body upon his death." We informed the patient and family about surgery hazards, while analytical therapy allowed the group to evaluate the actual wish for surgery. When the wish was brought up, the role of death urged the group to rethink the course of treatment. During the process, it became clear that the patient's desire for surgery, more than a wish for changing the genitalia, expressed an impulse related to issues of endorsement and acceptance of his male identity. This report raises interesting questions about sexuality in a social context and prompts the idea that sexuality is broader than sex itself, raising new questions on the psychological risks faced when considering a body change after years of living with a disorder of sex development.

  16. Androgens and cognitive abilities: Mental rotations skills and handedness in adult females with congenital adrenal hyperplasia

    DEFF Research Database (Denmark)

    Ripa, Caroline P.L.; Johannsen, T.H.; Mortensen, E.L.

    2006-01-01

    Research on animal and human populations has suggested elevated spatial abilities as well as higher incidence of left-handedness in genetic females exposed to abnormally high androgen levels perinatally. However, findings in humans are inconsistent. We administered the Mental Rotations Test......-wise matched on gender, age and school education were identified on either test. However, non-significant tendencies in the predicted direction on both inventories could be related to higher prenatal androgen levels in probands. Further analyses will reveal the relevance of this hypothesis in the present...

  17. Long-term outcome of genital reconstruction of Middle Eastern women with congenital adrenal hyperplasia

    Directory of Open Access Journals (Sweden)

    Raouf M Seyam

    2013-01-01

    Conclusions: CAH has a significant impact on adult women in our region. Most of the patients remain single. Few women get married and these are able to lead a nearly normal sexual life and give birth to healthy children.

  18. Behavioural Outcome in Children with Congenital Adrenal Hyperplasia: Experience of a Single Centre

    Directory of Open Access Journals (Sweden)

    Arini Nuran Idris

    2014-01-01

    Full Text Available The aim of this study was to determine the behavioral outcome in children with CAH and to identify the risk factors that may influence it. Participants (aged 6–18 years included 29 girls and 20 boys with CAH and unaffected siblings (25 girls and 17 boys. Psychological adjustment was assessed with parent reports on the Child Behavior Checklist (CBCL. Information about disease characteristics was obtained from medical records. Our study reveals that there was higher incidence of parent-reported problem of anxious/depressed and withdrawn/depressed behaviours, somatic complaints, social, thought, and attention problems, and rule-breaking, aggressive, internalizing, and externalizing behaviour among children with CAH compared to controls. The prevalence of internalizing behaviour problems was higher in CAH boys compared with that of controls. Psychosocial adjustment of girls with CAH was found to be similar to unaffected female controls and was within the normal population range. Family income may be associated with behavioral outcome. Glucocorticoid dose may reflect disease severity which may be associated with behavioral outcome. We conclude that internalizing behavioral problem was prevalent among boys with CAH reflecting maladaptive adjustment in coping with chronic illness. This highlighted the importance of psychological and social support for the patients and their families.

  19. Nocturia and benign prostatic hyperplasia

    Directory of Open Access Journals (Sweden)

    Laketić Darko

    2008-01-01

    Full Text Available Background/Aim. Nocturia often occurs in patients with benign prostate hyperplasia (BPH. The aim of the study was to investigate the frequency of nocturia in patients with BPH. Nocturia and other factors associated with it were also investigated. Methods. Forty patients with the confirmed diagnosis of BPH were studied. Transurethral and transvesical prostatectomy were performed in all the patients. Symptoms were evaluated with the International Prostate Symptom Score before, as well as three and six months after the surgery. All the results were compared with the control group. Results. There was no statistically significant difference between the patients before and after the surgery regarding nocturia. There was, however, a statistically significant difference between the operated patients and the control group regarding nocturia, as well as a statistically significant correlation between noctruia and the age of the patients in both the investigated and the control group. A correlation also existed between nocturia and the prostatic size. Conclusion. There was no statistically significant improvement in symptoms of nocturia after the surgery. It is necessary to be very careful in decision making in patients with nonabsolute indiction for surgery and isolated bothersome symptom of nocturia. Age of a patient should also be considered in the evaluation of favorable result of the surgery because of a significant correlation between noctura and the age of a patient.

  20. The state of Hawking radiation is non-classical

    Science.gov (United States)

    Brustein, Ram; Medved, A. J. M.; Zigdon, Yoav

    2018-01-01

    We show that the state of the Hawking radiation emitted from a large Schwarzschild black hole (BH) deviates significantly from a classical state, in spite of its apparent thermal nature. For this state, the occupation numbers of single modes of massless asymptotic fields, such as photons, gravitons and possibly neutrinos, are small and, as a result, their relative fluctuations are large. The occupation numbers of massive fields are much smaller and suppressed beyond even the expected Boltzmann suppression. It follows that this type of thermal state cannot be viewed as classical or even semiclassical. We substantiate this claim by showing that, in a state with low occupation numbers, physical observables have large quantum fluctuations and, as such, cannot be faithfully described by a mean-field or by a WKB-like semiclassical state. Since the evolution of the BH is unitary, our results imply that the state of the BH interior must also be non-classical when described in terms of the asymptotic fields. We show that such a non-classical interior cannot be described in terms of a semiclassical geometry, even though the average curvature is sub-Planckian.

  1. Statistical methods in quantum optics 2 non-classical fields

    CERN Document Server

    Carmichael, H J

    2007-01-01

    Statistical Methods in Quantum Optics 2 - Non-Classical Fields continues the development of the methods used in quantum optics to treat open quantum systems and their fluctuations. Its early chapters build upon the phase-space methods introduced in the first volume Statistical Methods in Quantum Optics 1 - Matter Equations and Fokker-Planck Equations: the difficulties these methods face in treating non-classical light are exposed, where the regime of large fluctuations – failure of the system size expansion – is shown to be particularly problematic. Cavity QED is adopted as a natural vehicle for extending quantum noise theory into this regime. In response to the issues raised, the theory of quantum trajectories is presented as a universal approach to the treatment of fluctuations in open quantum systems. This book presents its material at a level suitable for beginning researchers or students in an advanced course in quantum optics, or a course in quantum mechanics or statistical physics that deals with o...

  2. Adrenal failure due to bilateral adrenal metastasis of rectal cancer: A case report

    Directory of Open Access Journals (Sweden)

    Yuki Imaoka

    2017-01-01

    Conclusion: The incidence of adrenal insufficiency may be underestimated in patients with multiple metastasis. Appropriate therapy with adrenal corticosteroid hormone supplementation may lead to a significant improvement in the patient’s symptoms and quality of life.

  3. Mature cystic teratoma involving adrenal gland.

    Science.gov (United States)

    Bedri, Shahinaz; Erfanian, Kamil; Schwaitzberg, Steven; Tischler, Arthur S

    2002-01-01

    A mature cystic teratoma presented as an adrenal mass in a 57-yr-old woman. The tumor was found to be predominantly paraadrenal but focally interrupted the adrenal cortex so that an intraadrenal origin could not be ruled out. Similar lesions have been reported extremely rarely and should be considered in the differential diagnosis of hormonally silent adrenal tumors. The findings of rimlike calcification and fatty density on computed tomography may be helpful diagnostically.

  4. Ultrasonographi assessment of congenital adrenal masses

    International Nuclear Information System (INIS)

    Muro Velilla, D.; Sanguesa, C.; Alberto, C.; Lopez, A., Benlloch, C.

    1996-01-01

    The demonstrate the utility of ultrasound (US) in the initial assessment and follow-up of newborns with adrenal masses. A series of 21 newborns presenting adrenal mass studied on the basis of US findings, clinical assessment and biochemical data. Seven patients had congenital neuroblastoma, two had a benign tumor and twelve presented adrenal hemorrhage. Postnatal US study of the course of these patients is essential for the differential diagnosis of their lesions when not diagnosed prenatally. (Author) 20 refs

  5. Insuficiencia suprarrenal primaria por adrenalitis autoimnume

    OpenAIRE

    Muzzo B,Santiago; Izquierdo C,Gianina; Verbeke P,Sandra

    2002-01-01

    We report a 10 years old boy, admitted with a history of asthenia, anorexia and weight loss of 4 kg. Initial laboratory work up showed metabolic acidosis and hyponatremia. The patient had no circadian rhythm of serum cortisol and an adrenal stimulation test confirmed the presence of adrenal insufficiency. Anti-adrenal antibodies were positive. Treatment with cortisol and fluorocortisone resulted in a complete remission of symptoms (Rev Méd Chile 2002; 130: 901-6).

  6. Severe bilateral adrenal hemorrhages in a newborn complicated by persistent adrenal insufficiency

    OpenAIRE

    Zessis, Nicholas R; Nicholas, Jennifer L; Stone, Stephen I

    2018-01-01

    Summary Bilateral adrenal hemorrhages rarely occur during the neonatal period and are often associated with traumatic vaginal deliveries. However, the adrenal gland has highly regenerative capabilities and adrenal insufficiency typically resolves over time. We evaluated a newborn female after experiencing fetal macrosomia and a traumatic vaginal delivery. She developed acidosis and acute renal injury. Large adrenal hemorrhages were noted bilaterally on ultrasound, and she was diagnosed with a...

  7. Adrenal androgens and the menopausal transition.

    Science.gov (United States)

    Lasley, Bill L; Crawford, Sybil; McConnell, Daniel S

    2011-09-01

    The concept that adrenal androgen production gradually declines with age has changed after analysis of longitudinal data from the Study of Women's Health Across the Nation (SWAN). It is now recognized that 4 adrenal androgens rise during the menopausal transition in most women. Ethnic and individual differences in sex steroids are more apparent in circulating adrenal steroids than in either estradiol or cyclic ovarian steroid hormone profiles, particularly during the early and late perimenopause. Thus, adrenal steroid production may play a larger role in the occurrence of symptoms and the potential for healthier aging than previously recognized.

  8. The Lateralizing Asymmetry of Adrenal Adenomas

    Science.gov (United States)

    Hao, Meng; Lopez, Diana; Luque-Fernandez, Miguel Angel; Cote, Kathryn; Newfield, Jessica; Connors, Molly; Vaidya, Anand

    2018-01-01

    Abstract Context It is presumed that the incidence of adrenal adenomas is symmetric between the left and right adrenal gland; however, anecdotal observations suggest a potential lateralizing asymmetry. Objective To investigate the symmetry in detection of adrenal adenomas and relevance to patient care. Design Cross-sectional and longitudinal studies. Population and Setting One thousand three hundred seventy-six patients with abdominal computed tomography or magnetic resonance imaging demonstrating benign-appearing adrenal adenomas. Main Outcome Location and size of adrenal adenomas. Results Left-sided adenomas were discovered in 65% of patients, right-sided in 21%, and bilateral adenomas in 14%. Among unilateral adenomas, 75% were left-sided. Left-sided adenomas were more prevalent than right-sided adenomas in each size category except the largest: Adrenal adenomas are substantially more likely to be identified on the left adrenal than the right. This observation may be due to detection bias attributed to the location of the right adrenal, which may preclude identification of right-sided adenomas until they are substantially larger. These findings suggest the potential for an underrecognition of right-sided adenomas that may also impair the accurate detection of bilateral adrenal diseases. PMID:29644340

  9. Influence of Pasteurella multocida and high and low environmental temperatures on adrenals and bursa of Fabricius in turkeys

    Energy Technology Data Exchange (ETDEWEB)

    Simensen, E.; Olson, L.D.; Hahn, G.L.

    1980-01-01

    The morphologic changes in the adrenals and bursa of Fabricius were evaluated from turkeys inoculated with Pasteurella multocida either in the palatine air spaces or via drinking water and maintained at high (33.4-37.4 C), low (2.6-5.3 C), and moderate (19.8-22.4 C) temperatures in temperature-controlled chambers. There was a slight hyperplasia of the adrenal cortical cells and a hypertrophy of the nuclei in the uninoculated turkeys maintained at both high and low temperatures, but these changes were more marked in turkeys maintained at low temperatures. Regardless of the temperature to which the turkeys were exposed, there was an increase in adrenal weight, hyperplasia of the cortical cells, hypertrophy of the nuclei of the cortical cells, and depletion of lipid in the cortical cells in the turkeys that became depressed after inoculation with P. multocida. In the uninoculated turkeys exposed to high temperatures there was a reduction in the weight of the bursa of Fabricius, atrophy of the follicles, and a reduction in the number of lymphocytes within the follicle, which did not occur in the bursae from uninoculated turkeys maintained at low temperatures. In the turkeys inoculated with P. multocida, there was a marked reduction in bursal weight, atrophy of the follicles, and reduction in the number of lymphocytes within the follicles.

  10. Adrenal scan in 17-alpha-hydroxylase deficiency: false indication of adrenal adenoma

    International Nuclear Information System (INIS)

    Shore, R.M.; Lieberman, L.M.; Newman, T.J.; Friedman, A.; Bargman, G.J.

    1981-01-01

    A patient who was thought to have testicular feminization syndrome and primary aldosteronism had an adrenal scan that suggested an adrenal adenoma. After later diagnosis of 17-alpha-hydroxylase deficiency, she was treated with glucocorticoids rather than surgery. Her clinical course and a repeat adrenal scan confirmed she did not have a tumor

  11. Mammary fibroadenomatoid hyperplasia in a heifer

    Directory of Open Access Journals (Sweden)

    Lorena Ferreira Silva

    Full Text Available ABSTRACT: This manuscript described the anatomopathological and immunohistochemical findings in a rare case of mammary fibroadenomatoid hyperplasia in a 12-month-old Holstein heifer. A yellow, multilobulated, firm 20cm x 9cm x 6.5cm mass affecting the right quarters of the udder was observed. Total mastectomy was performed. Microscopic evaluation revealed severe hyperplasia of the mammary epithelium and numerous well-differentiated and mildly pleomorphic acini. Additionally, moderate proliferation of the fibrous connective tissue and the myoepithelial cells near the proliferating acini was evident. About 50% of the proliferating epithelial cells showed positive nuclear labeling for estrogen and progesterone receptors, and approximately one-third were positive for Ki-67. In addition, the myoepithelial cells exhibited diffuse nuclear immunoreactivity for p63. Based on the clinical, morphologic, and immunohistochemical findings, a diagnosis of mammary fibroadenomatoid hyperplasia with probable influence of ovarian steroids was made.

  12. Adrenal Castleman's disease mimicking other adrenal neoplasms: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Hong, Seung Baek; Lee, Nam Kyung; Kim, Suk; Han, Ga Jin; Ha, Hong Koo; Ku, Ja Yoon; Ahn, Sang Jeong; Lee, Chang Hun [Pusan National University Hospital, Pusan National University School of Medicine, Busan (Korea, Republic of)

    2017-01-15

    We present a rare case of adrenal Castleman's disease with hyaline vascular type mimicking other adrenal neoplasms in a 65-year-old woman. Although rare, the hyaline vascular type of adrenal Castleman's disease should be included in the differential diagnosis if an adrenal mass shows a well-defined, highly enhancing solid adrenal mass with peripheral rim enhancement, multiple satellite lymph nodes, and peritoneal thickening around the dominant mass on computed tomography as shown in this patient.

  13. Neoplasia versus hyperplasia of the retinal pigment epithelium

    DEFF Research Database (Denmark)

    Heegaard, Steffen; Larsen, J.N.B.; Fledelius, Hans C.

    2001-01-01

    ophthalmology, retinal pigment epithelium, adenoma, tumor-like hyperplasia, histology, immunohistochemistry, tumor, neoplasm, ultrasonography......ophthalmology, retinal pigment epithelium, adenoma, tumor-like hyperplasia, histology, immunohistochemistry, tumor, neoplasm, ultrasonography...

  14. A multiscale transport model for non-classical nanochannel electroosmosis

    Science.gov (United States)

    Bhadauria, Ravi; Aluru, N. R.

    2017-12-01

    We present a multiscale model describing the electroosmotic flow (EOF) in nanoscale channels involving high surface charge liquid-solid interfaces. The departure of the EOF velocity profiles from classical predictions is explained by the non-classical charge distribution in the confined direction including charge inversion, reduced mobility of interfacial counter-ions, and subsequent enhancement of the local viscosity. The excess component of the local solvent viscosity is modeled by the local application of the Fuoss-Onsager theory and the Hubbard-Onsager electro-hydrodynamic equation based dielectric friction theory. The electroosmotic slip velocity is estimated from the interfacial friction coefficient, which in turn is calculated using a generalized Langevin equation based dynamical framework. The proposed model for local viscosity enhancement and EOF velocity shows good agreement of corresponding physical quantities against relevant molecular dynamics simulation results, including the cases of anomalous transport such as EOF reversal.

  15. Non-Classical Smoothening of Nano-Scale Surface Corrugations

    Energy Technology Data Exchange (ETDEWEB)

    Aziz, Michael J.; Chason, Eric; Erlebacher, Jonah; Floro, Jerrold A.; Sinclair, Michael B.

    1999-05-20

    We report the first experimental observation of non-classical morphological equilibration of a corrugated crystalline surface. Periodic rippled structures with wavelengths of 290-550 nm were made on Si(OO1) by sputter rippling and then annealed at 650 - 750 °C. In contrast to the classical exponential decay with time, the ripple amplitude, A{lambda}(t), followed an inverse linear decay, A{lambda}(t)= A{lambda}(0)/(1 +k{lambda}t), agreeing with a prediction of Ozdemir and Zangwill. We measure the activation energy for surface relaxation to be 1.6±0.2 eV, consistent with an interpretation that dimers mediate transport.

  16. Highly Nonclassical Quantum States and Environment Induced Decoherence

    Science.gov (United States)

    Foldi, Peter

    2004-06-01

    In this thesis concrete quantum systems are investigated in the framework of the environment induced decoherence. The focus is on the dynamics of highly nonclassical quantum states, the Wigner function of which are negative over some regions of their domains. One of the chosen physical systems is a diatomic molecule, where the potential energy of the nuclei is an anharmonic function of their distance. A system of two-level atoms, which can be important from the viewpoint of quantum information technology, is also investigated. A method is described that is valid in both systems and can determine the characteristic time of the decoherence in a dynamical way. The direction of the decoherence and its relation to energy dissipation is also studied. Finally, a scheme is proposed that can prepare decoherence-free states using the experimental techniques presently available.

  17. On-Chip Architecture for Self-Homodyned Nonclassical Light

    Science.gov (United States)

    Fischer, Kevin A.; Kelaita, Yousif A.; Sapra, Neil V.; Dory, Constantin; Lagoudakis, Konstantinos G.; Müller, Kai; Vučković, Jelena

    2017-04-01

    In the last decade, there has been remarkable progress on the practical integration of on-chip quantum photonic devices, yet quantum-state generators remain an outstanding challenge. Simultaneously, the quantum-dot photonic-crystal-resonator platform has demonstrated a versatility for creating nonclassical light with tunable quantum statistics thanks to a newly discovered self-homodyning interferometric effect that preferentially selects the quantum light over the classical light when using an optimally tuned Fano resonance. In this work, we propose a general structure for the cavity quantum electrodynamical generation of quantum states from a waveguide-integrated version of the quantum-dot photonic-crystal-resonator platform, which is specifically tailored for preferential quantum-state transmission. We support our results with rigorous finite-difference time-domain and quantum-optical simulations and show how our proposed device can serve as a robust generator of highly pure single- and even multiphoton states.

  18. Non-Classical Smoothening of Nano-Scale Surface Corrugations

    International Nuclear Information System (INIS)

    Aziz, Michael J.; Chason, Eric; Erlebacher, Jonah; Floro, Jerrold A.; Sinclair, Michael B.

    1999-01-01

    We report the first experimental observation of non-classical morphological equilibration of a corrugated crystalline surface. Periodic rippled structures with wavelengths of 290-550 nm were made on Si(OO1) by sputter rippling and then annealed at 650 - 750 ampersand deg;C. In contrast to the classical exponential decay with time, the ripple amplitude, A λ (t), followed an inverse linear decay, A λ (t)= A λ (0)/(1 +k λ t), agreeing with a prediction of Ozdemir and Zangwill. We measure the activation energy for surface relaxation to be 1.6 ampersand plusmn;0.2 eV, consistent with an interpretation that dimers mediate transport

  19. Nonclassicality by Local Gaussian Unitary Operations for Gaussian States

    Directory of Open Access Journals (Sweden)

    Yangyang Wang

    2018-04-01

    Full Text Available A measure of nonclassicality N in terms of local Gaussian unitary operations for bipartite Gaussian states is introduced. N is a faithful quantum correlation measure for Gaussian states as product states have no such correlation and every non product Gaussian state contains it. For any bipartite Gaussian state ρ A B , we always have 0 ≤ N ( ρ A B < 1 , where the upper bound 1 is sharp. An explicit formula of N for ( 1 + 1 -mode Gaussian states and an estimate of N for ( n + m -mode Gaussian states are presented. A criterion of entanglement is established in terms of this correlation. The quantum correlation N is also compared with entanglement, Gaussian discord and Gaussian geometric discord.

  20. Thymic hyperplasia - clinical course and imaging diagnostic

    International Nuclear Information System (INIS)

    Drebov, R.; Panov, M.; Totev, M.; Deliverski, T.; Tcandev, I.; Velkovski, I.

    2006-01-01

    The real thymic hyperplasia is benign disease sometimes simulating malignant tumours. The aim of this study is to analyse the clinical symptoms of real thymic hyperplasia and the results from imaging diagnostic based on our clinical material. Clinical material include 27 children, aged from two months to 15 years, admitted in department of thoracic surgery, for a period of 20 years (1985 - 2004). We retrospectively analyze the clinical signs and results from X-ray investigation, CT (Siemens Somatom DRG and Philips Secura) and echocardiography (Acuson TX, 5 and 7 MHz). We discuss the diagnostic value of different methods as well as typical and atypical findings. (authors)

  1. Pseudoepitheliomatous Hyperplasia in a Red Pigment Tattoo

    Science.gov (United States)

    Kazlouskaya, Viktoryia

    2015-01-01

    Red pigment tattoos are known to cause pseudoepitheliomatous hyperplasia in the skin, frequently simulating squamous cell carcinoma or keratoacanthoma. Herein, the authors present two additional cases of red pigment tattoo pseudoepitheliomatous hyperplasia in which they noted a lichenoid tissue reaction. They reviewed the previously published cases and observed a lichenoid reaction in the histopathological images similar to hypertrophic lichen planus. The authors suggest that these reactions might best be referred to as “lichenoid reaction with pseudoepitheliomatous hyperplasia” or “hypertrophic lichen planus-like reaction.” Accordingly, recognition of an inflammatory component may allow additional treatment options. PMID:26705448

  2. Non-classical structures of organic compounds: unusual stereochemistry and hypercoordination

    International Nuclear Information System (INIS)

    Minkin, Vladimir I; Minyaev, Ruslan M; Hoffmann, Roald

    2002-01-01

    Non-classical structures of organic compounds are defined as molecules containing non-tetrahedral tetracoordinate and/or hypercoordinate carbon atoms. The evolution of the views on this subject is considered and the accumulated theoretical and experimental data on the structures and dynamic transformations of non-classical organic compounds are systematised. It is shown that computational analysis using the methods and the software potential of modern quantum chemistry has now acquired high predictive capacity and is the most important source of data on the structures of non-classical compounds. The bibliography includes 227 references.

  3. Hypoadrenia following adrenal venography in Cushing's disease

    International Nuclear Information System (INIS)

    Goth, M.; Szilagy, G.; Irsy, G.; Szabolcs, I.; Berentey, E.; Molnar, F.; Magyar, E.

    1984-01-01

    Three patients with Cushing's disease are reported. In two patients the hyperfunction of the adrenal glands creased following selective adrenal venography, moreover hypoadrenia has developed. In the third case necrosis following venography was proved by surgery and confirmed by histology. Regarding the therapeutic possibilities of Cushing's diesease the use of this diagnostic procedure is taken into consideration but not recommended. (orig.)

  4. Rare primary retroperitoneal teratoma masquerading as adrenal ...

    African Journals Online (AJOL)

    J.M. Ratkal

    Abstract. Objectives: To present a rare case of Primary mature cystic teratoma of right adrenal gland in adult female with an aim to review the published literature. Materials and Methods: The case details of a lady presenting with vague upper abdominal pain and on investigation was found to have a right adrenal mass were ...

  5. Neonatal adrenal hemorrhage mimicking an acute scrotum.

    Science.gov (United States)

    Adorisio, O; Mattei, R; Ciardini, E; Centonze, N; Noccioli, B

    2007-02-01

    Twenty-two cases of scrotal hematoma caused by neonatal adrenal hemorrhage are reported in the literature and unnecessary surgical exploration was performed in nine (41%), suspecting testicular torsion. In this paper, we present a newborn male with right adrenal gland hemorrhage causing right scrotal swelling and discoloration of groin managed conservatively.

  6. Rare primary retroperitoneal teratoma masquerading as adrenal ...

    African Journals Online (AJOL)

    Objectives: To present a rare case of Primary mature cystic teratoma of right adrenal gland in adult female with an aim to review the published literature. Materials and Methods: The case details of a lady presenting with vague upper abdominal pain and on investigation was found to have a right adrenal mass were collected ...

  7. chronic dehydration affects hydroelectrolytic equilibrium and adrenal

    African Journals Online (AJOL)

    A. Dekar-Madoui

    2017-05-01

    May 1, 2017 ... ABSTRACT. Shortage of water supply is the most stressful condition that can meet man and animals. Since the adrenal gland plays a pivotal role in the stress response, the objective of this work is to study, in the male Wistar rat, the repercussions of chronic dehydration on adrenal gland structure compared ...

  8. A Rare Presentation of Primary Hyperparathyroidism with Concurrent Aldosterone-Producing Adrenal Carcinoma

    Directory of Open Access Journals (Sweden)

    Mario Molina-Ayala

    2015-01-01

    Full Text Available Aldosterone-producing adrenocortical carcinomas are an extremely rare cause of hyperaldosteronism (<1%. Coexistence of different endocrine tumors warrants additional screening for multiple endocrine neoplasia syndromes, especially in young patients with large or malignant masses. We present the case of a 40-year-old man with a history of hypertension that presented with an incidental left adrenal tumor during an ultrasound performed for nephrolithiasis. Biochemical assessment showed a mildly elevated calcium (11.1 mg/dL, high parathyroid hormone, and a plasma aldosterone concentration/plasma renin activity ratio of 124.5 (normal < 30, compatible with primary hyperparathyroidism with a concomitant primary hyperaldosteronism. A Tc99m-MIBI scintigraphy showed an abnormally increased tracer uptake in the right superior parathyroid and abdominal computed tomography confirmed a left adrenal tumor of 20 cm. The patient underwent parathyroidectomy and adrenalectomy with final pathology reports of parathyroid hyperplasia and adrenal carcinoma with biochemical remission of both endocrinopathies. He was started on chemotherapy, but the patient developed a frontal cortex and an arm metastasis and finally died less than one year later.

  9. [Neonatal adrenal hematoma: various modes of presentation].

    Science.gov (United States)

    Fadil, F-Z; Lehlimi, M; Chemsi, M; Habzi, A; Benomar, S

    2014-09-01

    Neonatal adrenal hematoma is a rare condition, most frequently caused by trauma. We report three cases of adrenal hematoma admitted to the Neonatology and Neonatal Intensive Care Unit in the A. Harouchi Children's Hospital, the Ibn Rushd University Hospital in Casablanca, Morocco, over a 2-year period from January 2011 to December 2012. The average age of these patients was 5 days. The clinical presentations were diverse; the most common manifestations were intense jaundice in one case, acute adrenal insufficiency in one case, and severe anemia in the other case. Abdominal ultrasonography was used to confirm the diagnosis and monitor adrenal hemorrhage in all the patients. Analysis of clinical, laboratory, and ultrasonography data showed a favorable prognosis in all the patients. Based on these observations, we discuss the risk factors, clinical presentations, progression and management of neonatal adrenal hemorrhage. Copyright © 2014. Published by Elsevier SAS.

  10. Benign prostatic hyperplasia: symptoms and objective interpretation

    DEFF Research Database (Denmark)

    Andersen, J T

    1991-01-01

    Considerable new knowledge about benign prostatic hyperplasia has been gained over the past two decades, particularly with regard to its natural history, hydrodynamic changes in the lower urinary tract, and the symptomatic and urodynamic results of treatment. A survey of the literature has been u...

  11. Pseudoepitheliomatous Hyperplasia in Oral Lesions: A Review.

    Science.gov (United States)

    Nayak, Vaidhehi Narayan; Uma, K; Girish, H C; Murgod, Sanjay; Shyamala, K; Naik, Ranajit B

    2015-09-01

    Pseudoepitheliomatous hyperplasia (PEH) is a histopathological reaction pattern to various stimuli, which includes trauma, infection, inflammation, neoplasia. It is seen as tongue like epithelial proliferation invading the connective tissue and should not be mistaken for squamous cell carcinoma (SCC). This review enlists oral lesions which exhibit PEH with a note on how to differentiate SCC from PEH.

  12. Angiolymphoid Hyperplasia with Eosinophilia: A Case Report

    Directory of Open Access Journals (Sweden)

    Paola Esteves

    2015-05-01

    Full Text Available Angiolymphoid hyperplasia with eosinophilia is a rare disease considered as being a vascular malformation resulting from a subjacent arteriovenous shunt. It affects mostly the head of women between 20 and 40 years old and may present spontaneous involution.

  13. Transurethral microwave thermotherapy in benign prostatic hyperplasia

    NARCIS (Netherlands)

    Minnee, P.; Debruyne, F. M.; de la Rosette, J. J.

    2000-01-01

    This article reviews the available literature and data on high-energy transurethral microwave therapy (TUMT) in the treatment of benign prostatic hyperplasia (BPH) causing lower urinary tract symptoms (LUTS). TUMT is a safe, durable, (1-hour) procedure, without the need for anesthesia. Emphasis is

  14. Urodynamic implications of benign prostatic hyperplasia

    DEFF Research Database (Denmark)

    Jensen, K M; Andersen, J T

    1990-01-01

    By the age of 60, about 70% of men have developed benign prostatic hyperplasia (BPH), and 85%-95% of these have symptomatic dysfunction of the lower urinary tract, 10%-20% undergoing prostatectomy. Although transurethral resection of the prostate is generally considered to be a safe and effective...

  15. Materials with complex behaviour II properties, non-classical materials and new technologies

    CERN Document Server

    Oechsner, Andreas

    2012-01-01

    This book reviews developments and trends in advanced materials and their properties; modeling and simulation of non-classical materials and new technologies for joining materials. Offers tools for characterizing and predicting properties and behavior.

  16. Sudden vanishing and reappearance of nonclassical effects: General occurrence of finite-time decays and periodic vanishings of nonclassicality and entanglement witnesses

    International Nuclear Information System (INIS)

    Bartkowiak, Monika; Miranowicz, Adam; Wang Xiaoguang; Liu Yuxi; Leonski, Wieslaw; Nori, Franco

    2011-01-01

    Analyses of phenomena exhibiting finite-time decay of quantum entanglement have recently attracted considerable attention. Such decay is often referred to as sudden vanishing (or sudden death) of entanglement, which can be followed by its sudden reappearance (or sudden rebirth). We analyze various finite-time decays (for dissipative systems) and analogous periodic vanishings (for unitary systems) of nonclassical correlations as described by violations of classical inequalities and the corresponding nonclassicality witnesses (or quantumness witnesses), which are not necessarily entanglement witnesses. We show that these sudden vanishings are universal phenomena and can be observed: (i) not only for two- or multimode but also for single-mode nonclassical fields, (ii) not solely for dissipative systems, and (iii) at evolution times which are usually different from those of sudden vanishings and reappearances of quantum entanglement.

  17. Pharmacological treatment of the benign prostatic hyperplasia

    International Nuclear Information System (INIS)

    Perez Guerra, Yohani; Molina Cuevas, Vivian; Oyarzabal Yera, Ambar; Mas Ferreiro, Rosa

    2011-01-01

    Benign prostatic hyperplasia is a common disease in over 50 years-old men consisting in uncontrolled and benign growth of prostatic gland that leads to lower urinary tract symptoms. The etiology of benign prostatic hyperplasia is multifactoral involving the increased conversion of testosterone in dihydrotestosterone by the prostatic 5α-reductase action, which brought about events that encourage the prostate growth (static component) and the increase of the bladder and prostate smooth muscle tone (dynamic component) regulated by the aα 1 -adrenoceptors (ADR). The pharmacological treatment of the benign prostatic hyperplasia includes the prostatic 5aα-reductase inhibitors, the aα 1 -adrenoreceptor blockers, their combined therapy and the phytotherapy. This paper was aimed at presenting the most relevant aspects of the pharmacology of drugs used for treating the benign prostatic hyperplasia, and providing elements to analyze their efficacy, safety and tolerability. To this end, a review was made of the different drugs for the treatment of this pathology and they were grouped according to their mechanism of action. Natural products were included as lipid extracts from Serenoa repens and Pygeum africanum as well as D-004, a lipid extract from Roystonea regia fruits, with proved beneficial effects on the main etiological factors of benign prostatic hyperplasia. D-004 is a prostatic 5a-reductase inhibitor, an aα 1 -adrenoceptor antagonist, aα 5-lipooxygenase inhibitor and has antioxidant action, all of which reveals a multifactoral mechanism. The results achieved till now indicate that D-004 is a safe and well-tolerated product

  18. Testing nonclassicality in multimode fields: A unified derivation of classical inequalities

    International Nuclear Information System (INIS)

    Miranowicz, Adam; Bartkowiak, Monika; Wang Xiaoguang; Liu Yuxi; Nori, Franco

    2010-01-01

    We consider a way to generate operational inequalities to test nonclassicality (or quantumness) of multimode bosonic fields (or multiparty bosonic systems) that unifies the derivation of many known inequalities and allows to propose new ones. The nonclassicality criteria are based on Vogel's criterion corresponding to analyzing the positivity of multimode P functions or, equivalently, the positivity of matrices of expectation values of, e.g., creation and annihilation operators. We analyze not only monomials but also polynomial functions of such moments, which can sometimes enable simpler derivations of physically relevant inequalities. As an example, we derive various classical inequalities which can be violated only by nonclassical fields. In particular, we show how the criteria introduced here easily reduce to the well-known inequalities describing (a) multimode quadrature squeezing and its generalizations, including sum, difference, and principal squeezing; (b) two-mode one-time photon-number correlations, including sub-Poisson photon-number correlations and effects corresponding to violations of the Cauchy-Schwarz and Muirhead inequalities; (c) two-time single-mode photon-number correlations, including photon antibunching and hyperbunching; and (d) two- and three-mode quantum entanglement. Other simple inequalities for testing nonclassicality are also proposed. We have found some general relations between the nonclassicality and entanglement criteria, in particular those resulting from the Cauchy-Schwarz inequality. It is shown that some known entanglement inequalities can be derived as nonclassicality inequalities within our formalism, while some other known entanglement inequalities can be seen as sums of more than one inequality derived from the nonclassicality criterion. This approach enables a deeper analysis of the entanglement for a given nonclassicality.

  19. Quantitative Characterization of Non-Classic Polarization of Cations on Clay Aggregate Stability

    Science.gov (United States)

    Hu, Feinan; Li, Hang; Liu, Xinmin; Li, Song; Ding, Wuquan; Xu, Chenyang; Li, Yue; Zhu, Longhui

    2015-01-01

    Soil particle interactions are strongly influenced by the concentration, valence and ion species and the pH of the bulk solution, which will also affect aggregate stability and particle transport. In this study, we investigated clay aggregate stability in the presence of different alkali ions (Li+, Na+, K+, and Cs+) at concentrations from10−5 to 10−1 mol L−1. Strong specific ion effects on clay aggregate stability were observed, and showed the order Cs+>K+>Na+>Li+. We found that it was not the effects of ion size, hydration, and dispersion forces in the cation–surface interactions but strong non-classic polarization of adsorbed cations that resulted in these specific effects. In this study, the non-classic dipole moments of each cation species resulting from the non-classic polarization were estimated. By comparing non-classic dipole moments with classic values, the observed dipole moments of adsorbed cations were up to 104 times larger than the classic values for the same cation. The observed non-classic dipole moments sharply increased with decreasing electrolyte concentration. We conclude that strong non-classic polarization could significantly suppress the thickness of the diffuse layer, thereby weakening the electric field near the clay surface and resulting in improved clay aggregate stability. Even though we only demonstrated specific ion effects on aggregate stability with several alkali ions, our results indicate that these effects could be universally important in soil aggregate stability. PMID:25874864

  20. About the nonclassicality of states defined by nonpositivity of the P-quasiprobability

    International Nuclear Information System (INIS)

    Wuensche, Alfred

    2004-01-01

    The definition of nonclassical states in quantum optics by the nonpositivity of their Glauber-Sudarshan quasiprobability P(α,α * )-P(q,p) is investigated and it is shown that it hides some serious problems. It leads to a subdivision of squeezed thermal states into classical and nonclassical states which is difficult to interpret physically by some qualitatively different behaviour of the states. Nonclassical states are found in arbitrarily small neighbourhoods of every classical state that is illustrated by a very artificial modified thermal state. The observability of the criterion in comparison to that for nonclassicality of states determined by the nearest Hilbert-Schmidt distance to a class of reference states is discussed. The behaviour of the nonclassicality of states in models of phase-insensitive processes of damping and amplification is investigated and it is found that every nonclassical state eventually makes a transition to a classical state. However, this is not specific for the negativities or singularities of the Glauber-Sudarshan quasiprobability and is found in similar form for other quasiprobabilities, for example, for the Wigner quasiprobability. We discuss in quite general form some defects of the Glauber-Sudarshan quasiprobability if compared with classical distribution functions over the phase space, in particular the failure of an earlier advertised superposition formula

  1. Quantitative characterization of non-classic polarization of cations on clay aggregate stability.

    Directory of Open Access Journals (Sweden)

    Feinan Hu

    Full Text Available Soil particle interactions are strongly influenced by the concentration, valence and ion species and the pH of the bulk solution, which will also affect aggregate stability and particle transport. In this study, we investigated clay aggregate stability in the presence of different alkali ions (Li+, Na+, K+, and Cs+ at concentrations from10-5 to 10-1 mol L-1. Strong specific ion effects on clay aggregate stability were observed, and showed the order Cs+>K+>Na+>Li+. We found that it was not the effects of ion size, hydration, and dispersion forces in the cation-surface interactions but strong non-classic polarization of adsorbed cations that resulted in these specific effects. In this study, the non-classic dipole moments of each cation species resulting from the non-classic polarization were estimated. By comparing non-classic dipole moments with classic values, the observed dipole moments of adsorbed cations were up to 104 times larger than the classic values for the same cation. The observed non-classic dipole moments sharply increased with decreasing electrolyte concentration. We conclude that strong non-classic polarization could significantly suppress the thickness of the diffuse layer, thereby weakening the electric field near the clay surface and resulting in improved clay aggregate stability. Even though we only demonstrated specific ion effects on aggregate stability with several alkali ions, our results indicate that these effects could be universally important in soil aggregate stability.

  2. Adrenal myelolipoma with hyperandrogenemia and schizophrenia

    Directory of Open Access Journals (Sweden)

    Liu N

    2018-02-01

    Full Text Available Ningning Liu,1,2,* Wenhua Zhang,3,* Jiangshan Tan,1,4 Jiaxin Zhou,1 Xiaopeng Yu,5 Ningxin Ren,5 Zhiqing Fang,3 Wei Jiao,3 Yidong Fan,3 Cheng Liu6 1School of Medicine, Shandong University, Jinan, China; 2Institute of Mental Health, Peking University, The Sixth Hospital, Peking University, Beijing, China; 3Department of Urology, Qilu Hospital, Shandong University, Jinan, China; 4State Key Laboratory of Cardiovascular Disease, FuWai Hospital, Peking Union Medical College and Chinese Academy Medical Science, Beijing, China; 5School of Basic Medicine Sciences, Shandong University, Jinan, China; 6Department of Urology, Peking University Third Hospital, Beijing, China *These authors contributed equally to this work Abstract: Adrenal myelolipoma with hyperandrogenemia is extremely rare. We report a case of a 26-year-old Chinese female with schizophrenia, who presented with a hormonally active tumor causing hyperandrogenemia. The mass was found by computerized tomography when she had her gynecologic examination for secondary amenorrhea, and it was confirmed to be an adrenal myelolipoma after a histopathological study. She was referred for a left adrenal laparoscopic excision, and the size of adrenal myelolipoma was found to be more than 10 cm. We report this case because large adrenal myelolipomas with hyperandrogenemia and schizophrenia are rare, and adrenal myelolipoma associated with hyperandrogenemia might be determined by the enzymes involved in the production of hormones. Keywords: adrenal, hyperandrogenemia, myelolipoma, schizophrenia

  3. Neonatal adrenal hemorrhage presenting as late onset neonatal jaundice

    OpenAIRE

    Qureshi, Umar Amin; Ahmad, Nisar; Rasool, Akhter; Choh, Suhail

    2009-01-01

    Clinical manifestations of adrenal hemorrhage vary depending on the degree and rate of hemorrhage, as well as the amount of adrenal cortex compromised by hemorrhage. We report here a case of neonatal adrenal hemorrhage that presented with late onset neonatal jaundice. The cause of adrenal hemorrhage was birth asphyxia.

  4. Adrenal Ewing's Sarcoma in an Elderly Man.

    Science.gov (United States)

    Toda, Kazuyoshi; Ishii, Sumiyasu; Yasuoka, Hidetoshi; Nishioka, Masaki; Kobayashi, Takayuki; Horiguchi, Kazuhiko; Tomaru, Takuya; Ozawa, Atsushi; Shibusawa, Nobuyuki; Satoh, Tetsurou; Koshi, Hiromi; Segawa, Atsuki; Shimizu, Shin-Ichi; Oyama, Tetsunari; Yamada, Masanobu

    2018-02-15

    Ewing's sarcoma usually arises in the bones of children and adolescents. We herein report a 74-year-old man with Ewing's sarcoma in the adrenal gland. The diagnosis was confirmed by a genetic test, pathological studies, and several imaging studies. He already had multiple liver metastases when he was transferred to our hospital and died on the 37th day. The diagnosis was further confirmed by autopsy studies. Adrenal Ewing's sarcoma is very rare, and our patient was older than other reported cases. Ewing's sarcoma should be considered even in elderly patients with adrenal tumors.

  5. Non-classical state engineering for quantum networks

    Energy Technology Data Exchange (ETDEWEB)

    Vollmer, Christina E.

    2014-01-24

    The wide field of quantum information processing and quantum networks has developed very fast in the last two decades. Besides the regime of discrete variables, which was developed first, the regime of continuous variables represents an alternative approach to realize many quantum applications. Non-classical states of light, like squeezed or entangled states, are a fundamental resource for quantum applications like quantum repeaters, quantum memories, quantum key distribution, quantum spectroscopy, and quantum metrology. These states can be generated successfully in the infrared wavelength regime. However, for some tasks other wavelengths, especially in the visible wavelength regime, are desirable. To generate non-classical states of light in this wavelength regime frequency up-conversion can be used, since all quantum properties are maintained in this process. The first part of this thesis deals with the experimental frequency up-conversion of quantum states. Squeezed vacuum states of light at 1550 nm were up-converted to 532 nm and a noise reduction of -1.5 dB at 532 nm was achieved. These states can be used for increasing the sensitivity of gravitational wave detectors or spectroscopic measurements. Furthermore, one part of an entangled state at 1550 nm was up-converted to 532 nm and, thus, entanglement between these two wavelengths was generated and characterized to -1.4 dB following Duan et al. With such a quantum link it is possible to establish a quantum network, which takes advantage of the low optical loss at 1550 nm for information transmission and of atomic transitions around 532 nm for a quantum memory in a quantum repeater. For quantum networks the distribution of entanglement and especially of a quantum key is essential. In the second part of this thesis the experimental distribution of entanglement by separable states is demonstrated. The underlying protocol requires a special three-mode state, which is separable in two of the three splittings. With

  6. Non-classical state engineering for quantum networks

    International Nuclear Information System (INIS)

    Vollmer, Christina E.

    2014-01-01

    The wide field of quantum information processing and quantum networks has developed very fast in the last two decades. Besides the regime of discrete variables, which was developed first, the regime of continuous variables represents an alternative approach to realize many quantum applications. Non-classical states of light, like squeezed or entangled states, are a fundamental resource for quantum applications like quantum repeaters, quantum memories, quantum key distribution, quantum spectroscopy, and quantum metrology. These states can be generated successfully in the infrared wavelength regime. However, for some tasks other wavelengths, especially in the visible wavelength regime, are desirable. To generate non-classical states of light in this wavelength regime frequency up-conversion can be used, since all quantum properties are maintained in this process. The first part of this thesis deals with the experimental frequency up-conversion of quantum states. Squeezed vacuum states of light at 1550 nm were up-converted to 532 nm and a noise reduction of -1.5 dB at 532 nm was achieved. These states can be used for increasing the sensitivity of gravitational wave detectors or spectroscopic measurements. Furthermore, one part of an entangled state at 1550 nm was up-converted to 532 nm and, thus, entanglement between these two wavelengths was generated and characterized to -1.4 dB following Duan et al. With such a quantum link it is possible to establish a quantum network, which takes advantage of the low optical loss at 1550 nm for information transmission and of atomic transitions around 532 nm for a quantum memory in a quantum repeater. For quantum networks the distribution of entanglement and especially of a quantum key is essential. In the second part of this thesis the experimental distribution of entanglement by separable states is demonstrated. The underlying protocol requires a special three-mode state, which is separable in two of the three splittings. With

  7. Adrenal Collision Tumor: Coexistence of Pigmented Adrenal Cortical Oncocytoma and Ganglioneuroma

    Directory of Open Access Journals (Sweden)

    Hye Seung Lee

    2016-01-01

    Full Text Available Background. Adrenal collision tumors (ACTs, in which distinct tumors coexist without intermingling in the same adrenal gland, are rare and their actual prevalence is unknown. ACTs commonly consist of adrenal cortical adenoma, pheochromocytoma, or metastatic malignant tumor. Case Report. A 32-year-old woman who had been experiencing gastric discomfort for one month was referred to our hospital with abnormal imaging findings. The physical examination and the laboratory data including endocrine studies were unremarkable. Abdomen computed tomography (CT and magnetic resonance imaging (MRI showed two adjacent masses in the left suprarenal fossa, and a laparoscopic left adrenalectomy was done. Histological and immunohistochemical (IHC examinations revealed two distinct tumors: a pigmented adrenal cortical oncocytoma (ACO and a ganglioneuroma, respectively. Conclusion. Both tumors are rare in the adrenal gland and exist as ACTs only exceptionally rarely. This is the first reported case of coexisting oncocytoma and ganglioneuroma in the same adrenal gland to our knowledge.

  8. Severe bilateral adrenal hemorrhages in  a  newborn complicated by persistent adrenal insufficiency

    Directory of Open Access Journals (Sweden)

    Nicholas R Zessis

    2018-02-01

    Full Text Available Bilateral adrenal hemorrhages rarely occur during the neonatal period and are often associated with traumatic vaginal deliveries. However, the adrenal gland has highly regenerative capabilities and adrenal insufficiency typically resolves over time. We evaluated a newborn female after experiencing fetal macrosomia and a traumatic vaginal delivery. She developed acidosis and acute renal injury. Large adrenal hemorrhages were noted bilaterally on ultrasound, and she was diagnosed with adrenal insufficiency based on characteristic electrolyte changes and a low cortisol (4.2 μg/dL. On follow-up testing, this patient was unable to be weaned off of hydrocortisone or fludrocortisone despite resolution of hemorrhages on ultrasound. Providers should consider bilateral adrenal hemorrhage when evaluating critically ill neonates after a traumatic delivery. In extreme cases, this may be a persistent process.

  9. Early diagnosis of and surgical strategy for adrenal medullary disease in MEN II gene carriers

    Energy Technology Data Exchange (ETDEWEB)

    Jansson, S.; Tisell, L.E.; Fjaelling, M.L.; Lindberg, S.; Jacobsson, L.; Zachrisson, B.F.

    1988-01-01

    Sixteen multiple endocrine neoplasia type II (MEN II) gene carriers--12 who had undergone thyroidectomy because of medullary carcinoma of the thyroid and 4 whose thyroid glands had been removed because of C cell hyperplasia--were examined for the presence of pheochromocytomas. No patient had sought medical advice for pheochromocytoma symptoms. Fourteen patients had MEN IIa syndromes, one patient had a MEN IIb and another patient had a mixed syndrome of von Recklinghausen's neurofibromatosis and MEN II. Eight patients had undergone unilateral adrenalectomy for pheochromocytoma 11 +/- 4 years before. The patients underwent clinical examination, determination of the urinary excretion of catecholamines and metabolites, and /sup 131/I-metaiodobenzylguanidine (/sup 131/I-MIBG) and CAT scans. /sup 131/I-MIBG scanning was performed with images 1, 4, and 7 days after the radionuclide injection. In seven of eight patients who had undergone unilateral adrenalectomies, the /sup 131/I-MIBG scans showed accumulation of the radionuclide in the remaining adrenal gland. Bilateral adrenal accumulation of the radionuclide was demonstrated in seven of eight MEN IIa gene carriers who had not undergone adrenalectomy. Five patients, two of whom had undergone adrenalectomy, were found to have unilateral pheochromocytomas less than 2 cm in diameter. Only one of these five patients had an elevated excretion of urinary catecholamines. Between day 4 and day 7 after /sup 131/I-MIBG injection, adrenal glands with pheochromocytomas increased their relative accumulation of the radionuclide significantly more (p less than 0.02) than did adrenal glands without any demonstrable pheochromocytomas. All the pheochromocytomas were viewed by means of CAT scans.

  10. Whole Exome Sequencing for the differential diagnosis of primary adrenal insufficiency in children

    Directory of Open Access Journals (Sweden)

    Li F Chan

    2015-08-01

    Full Text Available Adrenal insufficiency is a rare, but potentially fatal medical condition. In children the cause is most commonly congenital and in recent years a growing number of causative gene mutations have been identified resulting in a myriad of syndromes that share adrenal insufficiency as one of the main characteristics. The evolution of adrenal insufficiency is dependent on the variant and the particular gene affected, meaning rapid and accurate diagnosis is imperative for effective treatment of the patient. Common practice is for candidate genes to be sequenced individually, which is a time consuming process and complicated by overlapping clinical phenotypes. However, with the availability, and increasing cost effectiveness of whole exome sequencing there is the potential for this to become a powerful diagnostic tool. Here we report the results of whole exome sequencing of 43 patients referred to us with a diagnosis of familial glucocorticoid deficiency (FGD who were mutation negative for MC2R, MRAP and STAR the most commonly mutated genes in FGD. WES provided a rapid genetic diagnosis in 17/43 sequenced patients, for the remaining 60% the gene defect may be within intronic/regulatory regions not covered by WES or may be in gene(s representing novel aetiologies. The diagnosis of isolated or familial glucocorticoid deficiency was only confirmed in 3 of the 17 patients, other genetic diagnoses were adrenal hypo- and hyperplasia, Triple A and autoimmune polyendocrinopathy syndrome type I, emphasizing both the difficulty of phenotypically distinguishing between disorders of PAI and the utility of WES as a tool to achieve this.

  11. Adrenal pathology in childhood: a spectrum of disease

    Energy Technology Data Exchange (ETDEWEB)

    Paterson, Anne [Royal Belfast Hospital for Sick Children, 180 Falls Road, Belfast BT12 6BE (United Kingdom)

    2002-10-01

    Adrenal lesions in children may present with an asymptomatic adrenal mass lesion, an endocrinopathy, a hypertensive or metabolic crisis or a paraneoplastic syndrome. Some of the underlying disease processes remain localised within the adrenal gland or may develop into disseminated disease. The role of the radiologist is to confirm the adrenal location of a lesion and to document any other foci of disease. Ultrasound, CT, MR imaging and metaiodobenzylguanidine scintigraphy are the most commonly utilised imaging modalities for examining the adrenal glands and their pathologies in children. This paper reviews the spectrum of adrenal disease in childhood and presents the information as a series of radiological appearances: anomalies of adrenal shape, location, number and size, cystic adrenal masses, solid adrenal mass lesions, and adrenal calcifications are all examined. The radiological findings must always be interpreted in conjunction with the patient's age, the clinical history, the findings on physical examination and the available biochemical data. (orig.)

  12. Bilateral adrenal histoplasmosis in an immunocompetent man from Texas

    Directory of Open Access Journals (Sweden)

    Colin J. Rog

    2016-12-01

    Full Text Available Disseminated histoplasmosis affecting the adrenal gland(s of immunocompetent adults is a very rare infection. Here, we present a case of bilateral adrenal histoplasmosis in an immunocompetent, 62-year-old gentleman from Texas along with a brief review of the published literature. Given the risk of patient decompensation secondary to adrenal insufficiency and the wide availability of effective treatments, adrenal histoplasmosis must be considered even in immunocompetent adults who acquire adrenal masses.

  13. Persistence of histoplasma in adrenals 7 years after antifungal therapy

    Directory of Open Access Journals (Sweden)

    Deepak Kothari

    2013-01-01

    Full Text Available Adrenal histoplasmosis is an uncommon cause for adrenal insufficiency. The duration of treatment for adrenal histoplasmosis is not clear. Existing treatment regimens advocate antifungals given for periods ranging from 6 months to 2 years. We report here a rare case who showed persistence of histoplasma in adrenal biopsy 7 years after being initially treated with itraconazole for 9 months. This calls for a prolonged therapy with regular review of adrenal morphology and histology in these patients.

  14. Bilateral adrenal histoplasmosis in an immunocompetent man from Texas.

    Science.gov (United States)

    Rog, Colin J; Rosen, Daniel G; Gannon, Francis H

    2016-12-01

    Disseminated histoplasmosis affecting the adrenal gland(s) of immunocompetent adults is a very rare infection. Here, we present a case of bilateral adrenal histoplasmosis in an immunocompetent, 62-year-old gentleman from Texas along with a brief review of the published literature. Given the risk of patient decompensation secondary to adrenal insufficiency and the wide availability of effective treatments, adrenal histoplasmosis must be considered even in immunocompetent adults who acquire adrenal masses.

  15. Adrenal insufficiency due to primary bilateral adrenal non-Hodgkin's lymphoma.

    Science.gov (United States)

    Hamid Zargar, A; Ahmad Laway, B; Alam Bhat, K; Shah, A; Ahmad, M; Aejaz Aziz, S; Iftikhar Bashir, M; Iqbal Wani, A; Hayat Bhat, M

    2004-09-01

    A case of primary adrenal insufficiency, secondary to primary bilateral adrenal lymphoma is reported. A 50-year-old woman presented with features of primary adrenal insufficiency (darkening of skin, asthenia, anorexia, constipation) for at least 8 months. Clinical examination was unremarkable except for low body mass index and generalized skin and buccal mucosal pigmentation. Routine investigations including complete hemogram, serum chemistry, urine analysis, chest radiograph and electrocardiogram were normal; serum lactate dehydrogenase was moderately elevated. Primary adrenal insufficiency was confirmed on cortisol dynamics (very low basal and peak cortisol) after insulin-induced hypoglycemia. Routinely detected adrenal masses on ultrasonography were confirmed by contrast enhanced CT abdomen. A diagnosis of primary adrenal non- Hodgkin's lymphoma (B-cell) was made after exploratory laprotomy and further staging. The patient was put on combination chemotherapy (CHOP) protocol, but was lost to follow-up after receiving two cycles of treatment. Primary adrenal lymphoma, although a rare entity, needs to be suspected in patients with features of primary adrenal insufficiency who have evidence of bilateral adrenal masses on imaging.

  16. Nonlinear Quantum Optical Springs and Their Nonclassical Properties

    International Nuclear Information System (INIS)

    Faghihi, M.J.; Tavassoly, M.K.

    2011-01-01

    The original idea of quantum optical spring arises from the requirement of quantization of the frequency of oscillations in the Hamiltonian of harmonic oscillator. This purpose is achieved by considering a spring whose constant (and so its frequency) depends on the quantum states of another system. Recently, it is realized that by the assumption of frequency modulation of ω to ω√1+μa † a the mentioned idea can be established. In the present paper, we generalize the approach of quantum optical spring with particular attention to the dependence of frequency to the intensity of radiation field that naturally observes in the nonlinear coherent states, from which we arrive at a physical system has been called by us as nonlinear quantum optical spring. Then, after the introduction of the generalized Hamiltonian of nonlinear quantum optical spring and it's solution, we will investigate the nonclassical properties of the obtained states. Specially, typical collapse and revival in the distribution functions and squeezing parameters, as particular quantum features, will be revealed. (electromagnetism, optics, acoustics, heat transfer, classical mechanics, and fluid dynamics)

  17. Rodent Models of Non-classical Progesterone Action Regulating Ovulation

    Directory of Open Access Journals (Sweden)

    Melinda A. Mittelman-Smith

    2017-07-01

    Full Text Available It is becoming clear that steroid hormones act not only by binding to nuclear receptors that associate with specific response elements in the nucleus but also by binding to receptors on the cell membrane. In this newly discovered manner, steroid hormones can initiate intracellular signaling cascades which elicit rapid effects such as release of internal calcium stores and activation of kinases. We have learned much about the translocation and signaling of steroid hormone receptors from investigations into estrogen receptor α, which can be trafficked to, and signal from, the cell membrane. It is now clear that progesterone (P4 can also elicit effects that cannot be exclusively explained by transcriptional changes. Similar to E2 and its receptors, P4 can initiate signaling at the cell membrane, both through progesterone receptor and via a host of newly discovered membrane receptors (e.g., membrane progesterone receptors, progesterone receptor membrane components. This review discusses the parallels between neurotransmitter-like E2 action and the more recently investigated non-classical P4 signaling, in the context of reproductive behaviors in the rodent.

  18. Multi-crack imaging using nonclassical nonlinear acoustic method

    Science.gov (United States)

    Zhang, Lue; Zhang, Ying; Liu, Xiao-Zhou; Gong, Xiu-Fen

    2014-10-01

    Solid materials with cracks exhibit the nonclassical nonlinear acoustical behavior. The micro-defects in solid materials can be detected by nonlinear elastic wave spectroscopy (NEWS) method with a time-reversal (TR) mirror. While defects lie in viscoelastic solid material with different distances from one another, the nonlinear and hysteretic stress—strain relation is established with Preisach—Mayergoyz (PM) model in crack zone. Pulse inversion (PI) and TR methods are used in numerical simulation and defect locations can be determined from images obtained by the maximum value. Since false-positive defects might appear and degrade the imaging when the defects are located quite closely, the maximum value imaging with a time window is introduced to analyze how defects affect each other and how the fake one occurs. Furthermore, NEWS-TR-NEWS method is put forward to improve NEWS-TR scheme, with another forward propagation (NEWS) added to the existing phases (NEWS and TR). In the added phase, scanner locations are determined by locations of all defects imaged in previous phases, so that whether an imaged defect is real can be deduced. NEWS-TR-NEWS method is proved to be effective to distinguish real defects from the false-positive ones. Moreover, it is also helpful to detect the crack that is weaker than others during imaging procedure.

  19. Multi-crack imaging using nonclassical nonlinear acoustic method

    International Nuclear Information System (INIS)

    Zhang Lue; Zhang Ying; Liu Xiao-Zhou; Gong Xiu-Fen

    2014-01-01

    Solid materials with cracks exhibit the nonclassical nonlinear acoustical behavior. The micro-defects in solid materials can be detected by nonlinear elastic wave spectroscopy (NEWS) method with a time-reversal (TR) mirror. While defects lie in viscoelastic solid material with different distances from one another, the nonlinear and hysteretic stress—strain relation is established with Preisach—Mayergoyz (PM) model in crack zone. Pulse inversion (PI) and TR methods are used in numerical simulation and defect locations can be determined from images obtained by the maximum value. Since false-positive defects might appear and degrade the imaging when the defects are located quite closely, the maximum value imaging with a time window is introduced to analyze how defects affect each other and how the fake one occurs. Furthermore, NEWS-TR-NEWS method is put forward to improve NEWS-TR scheme, with another forward propagation (NEWS) added to the existing phases (NEWS and TR). In the added phase, scanner locations are determined by locations of all defects imaged in previous phases, so that whether an imaged defect is real can be deduced. NEWS-TR-NEWS method is proved to be effective to distinguish real defects from the false-positive ones. Moreover, it is also helpful to detect the crack that is weaker than others during imaging procedure. (electromagnetism, optics, acoustics, heat transfer, classical mechanics, and fluid dynamics)

  20. A Non-classical Hydrostatic Equation for Unsaturated Porous Media

    Science.gov (United States)

    Wang, J.; Bras, R. L.; Illangasekare, T. H.; Sakaki, T.; Detwiler, R. L.

    2013-12-01

    The classical hydrostatic equation (CHE) of a liquid fluid in unsaturated porous media is critically re-examined vis-a-vis Newton's first law, the principle of minimum potential energy, and the principle of virtual work. We found that the CHE is inconsistent with these fundamental principles. A non-classical hydrostatic equation (NCHE), derived based on Newton's first law and the principle of minimum potential energy, includes an extra term as a nonlinear function of tension and liquid content. The NCHE makes four quantitative and experimentally verifiable predictions that are inconsistent with the CHE: 1) tension head is a nonlinear function of elevation (nonlinearity); 2) the vertical profile of equilibrium liquid content extends within a finite distance bounded by 'edges' (discontinuity); 3) equilibrium profiles of liquid content and tension head may decrease or increase with elevation (non-monotonicity); and 4) opposite horizontal equilibrium distribution of liquid content to that according to the CHE (lateral reversion). Preliminary laboratory experiments have confirmed nonlinearity and provided suggestive evidence consistent with discontinuity. In addition, an analytical expression of areal liquid content as a function of liquid content in terms of retention curve was obtained for the first time. This study provides an alternative explanation of flow and transport in unsaturated porous media deviating from Darcy's law or Richards' equation, and may be a starting point of formulating revised Darcy's and Richards' equation.

  1. Systemic Lupus Erythematosus Presenting as Acute Adrenal ...

    African Journals Online (AJOL)

    hanumantp

    hereby report such a case of SLE presenting as acute adrenal insufficiency. ... Kidney function tests, Liver function tests, serum calcium, and ... renal involvement. Patient was successfully managed with steroids and improved clinically. Keywords: Addison's disease, Autoimmune diseases, Systemic lupus erythematosus.

  2. Adrenal Androgens and the Menopausal Transition

    OpenAIRE

    Lasley, B. L.; Crawford, S.; McConnell, D.S.

    2011-01-01

    The concept that adrenal androgen production gradually declines with age has changed following the analysis of the longitudinal data collected in the Study of Women’s Health Across the Nation (SWAN). It is now recognized that four adrenal androgens (3-beta hydroxy-5-androsten-17-one or dehydroepiandrosterone--DHEA, its sulfate, dehydroepiandrosterone sulfate--DHEAS; androst-4-ene, 3,17-dione or androstenedione; and androst-5-ene-3-beta, 17-beta diol, also known as androstenediol or Adiol) ris...

  3. Ultrasound diagnosis of adrenal hemorrhage in meningococcemia

    International Nuclear Information System (INIS)

    Sarnaik, A.P.; Sanfilippo, D.J.K.; Slovis, T.L.; Children's Hospital of Michigan, Detroit; Wayne State Univ., Detroit, MI

    1988-01-01

    Adrenal hemorrhage (AH) is a well-described complication of the neonatal period, anticoagulant therapy, and overwhelming bacterial infection especially with N. meningitis. Until recently the diagnosis of acute AH was based predominantly on autopsy findings. Ultrasound and computed tomography examinations have been successfully used for antemortem detection of AH in neonates and anticoagulated patients. We report two patients with fulminant meningococcal infection who demonstrated bilateral adrenal hemorrhages on ultrasonography. (orig.)

  4. Ultrasound diagnosis of adrenal hemorrhage in meningococcemia

    Energy Technology Data Exchange (ETDEWEB)

    Sarnaik, A.P.; Sanfilippo, D.J.K.; Slovis, T.L.

    1988-07-01

    Adrenal hemorrhage (AH) is a well-described complication of the neonatal period, anticoagulant therapy, and overwhelming bacterial infection especially with N. meningitis. Until recently the diagnosis of acute AH was based predominantly on autopsy findings. Ultrasound and computed tomography examinations have been successfully used for antemortem detection of AH in neonates and anticoagulated patients. We report two patients with fulminant meningococcal infection who demonstrated bilateral adrenal hemorrhages on ultrasonography.

  5. Chronic Heroin Dependence Leading to Adrenal Insufficiency

    Directory of Open Access Journals (Sweden)

    Gautam Das

    2014-01-01

    Full Text Available Opioids have been the mainstay for pain relief and palliation over a long period of time. They are commonly abused by drug addicts and such dependence usually imparts severe physiologic effects on multiple organ systems. The negative impact of opioids on the endocrine system is poorly understood and often underestimated. We describe a patient who developed severe suppression of the hypothalamic-pituitary adrenal (HPA axis leading to secondary adrenal insufficiency due to long standing abuse of opioids.

  6. Rough hypoplastic amelogenesis imperfecta with follicular hyperplasia.

    Science.gov (United States)

    Peters, E; Cohen, M; Altini, M

    1992-07-01

    This report documents a unique case of rough hypoplastic amelogenesis imperfecta with apparent anterior oligodontia and multiple anomalies of the associated mesenchymally derived tissues. Multiple unerupted teeth showed hypercementosis, distorted roots with aberrant dentin formation, and marked follicular hyperplasia. The hyperplastic follicles had a complex histopathologic appearance that recapitulated some features of the WHO-type odontogenic fibroma. The features of these teeth, the nature of the associated follicular lesions, and their relationship to the unerupted teeth are discussed.

  7. Estudio de 34 pacientes con incidentaloma suprarrenal A study of 34 cases of adrenal incidentaloma

    Directory of Open Access Journals (Sweden)

    Raúl A. Chervin

    2007-08-01

    clinical concern. Although in most cases they are benign and hormonally nonfunctioning, it is important to appropriately identify those few cases of malignant or hyperfunctioning lesions of surgical resolution. Although several proposals for an optimal diagnostic strategy have been designed, controversy over a series of questions still persists. In the present retrospective study we analyzed 34 patients with adrenal incidentaloma. Of these, 32% of the patients displayed hyperfunctioning pathologies that included subclinical Cushing's syndrome in four patients, probable primary hyperaldosteronism in two, late onset congenital adrenal hyperplasia in one, adrenocortical carcinoma in one and pheochromocytoma in three. CT and/or MRI permitted the identification of adenomas and were crucial to decide on surgery in two patients with nonfunctioning pheochromocytomas and in a patient carrying a primitive adrenocortical carcinoma, a diagnosis also suggested by a mixed pattern of hypersecretion of androgens and cortisol. In a diabetic and hypertensive patient with subclinical Cushing's syndrome both comorbidities were solved by surgery. Nonfunctioning tumors were mainly adenomas (87% with individual cases of histoplasmosis, pseudocyst, idiopathic adrenal hyperplasia and mielolipoma. Six of the eight operated patients presented malignant and/or hyperfunctioning tumors. The pathology associated with incidentalomas represents a broad spectrum of risk for patients and reaffirms the necessity for a meticulous clinical, biochemical, and imaging evaluation in order to make appropriate decisions.

  8. Denture hyperplasia with areas simulating oral inverted ductal papilloma.

    Science.gov (United States)

    Vargas, Pablo Agustin; Perez, Danyel Elias da Cruz; Jorge, Jacks; Rangel, Ana Lúcia Carrinho Ayrosa; León, Jorge Esquiche; Almeida, Oslei Paes de

    2005-07-01

    Denture hyperplasia is a reactive lesion of the oral mucosa, usually associated to an ill-fitting denture. This lesion is easily diagnosed and in some cases distinct microscopic variations such as osseous, oncocytic and squamous metaplasia may be found. These metaplastic alterations probably are associated with the lymphocytic infiltrate usually present in denture hyperplasia. We present a case of denture hyperplasia containing salivary gland tissue with ductal alterations mimicking an oral inverted ductal papilloma.

  9. Experimental detection of nonclassical correlations in mixed-state quantum computation

    International Nuclear Information System (INIS)

    Passante, G.; Moussa, O.; Trottier, D. A.; Laflamme, R.

    2011-01-01

    We report on an experiment to detect nonclassical correlations in a highly mixed state. The correlations are characterized by the quantum discord and are observed using four qubits in a liquid-state nuclear magnetic resonance quantum information processor. The state analyzed is the output of a DQC1 computation, whose input is a single quantum bit accompanied by n maximally mixed qubits. This model of computation outperforms the best known classical algorithms and, although it contains vanishing entanglement, it is known to have quantum correlations characterized by the quantum discord. This experiment detects nonvanishing quantum discord, ensuring the existence of nonclassical correlations as measured by the quantum discord.

  10. J(Si,H) Coupling Constants in Nonclassical Transition-Metal Silane Complexes.

    Science.gov (United States)

    Scherer, Wolfgang; Meixner, Petra; Batke, Kilian; Barquera-Lozada, José E; Ruhland, Klaus; Fischer, Andreas; Eickerling, Georg; Eichele, Klaus

    2016-09-12

    We will outline that the sign and magnitude of J(Si,H) coupling constants provide a highly sensitive tool to measure the extent of Si-H bond activation in nonclassical silane complexes. Up to now, this structure-property relationship was obscured by erroneous J(Si,H) sign determinations in the literature. These new findings also help to identify the salient control parameters of the Si-H bond activation process in nonclassical silane complexes. © 2016 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.

  11. Adrenal scintigraphy with 131I-19 iodocholesterol. Interest in Cushing syndrome investigation

    International Nuclear Information System (INIS)

    Vuidard, Claudine.

    1977-01-01

    A review of the physico-chemical properties of 131 I-19-iodocholesterol is followed by a study of its metabolism in both animals and man, giving for the latter the doses delivered to different organs and especially to the gonads. The exploration technique is then described. Scintigraphic examinations are performed on the 3rd day, the 8th day and sometimes the 15th day after injection. However the uptake rate at the adrenal gland depends essentially on the causes of the illness affecting the suprarenal. In Cushing's syndrome the uptake rate is relatively fast and the 1st scintigraphic examination must therefore be carried out earlier (48 hours after injection). The value of isotopic exploration in the etiological evaluation of hypercortisolism is shown to be chiefly morphological, the scintigraphic image serving as a guide to etiological diagnosis. If too adrenal glands are visible this is a sign of bilateral suprerenal hyperplasia. Only one gland clearly visible indicates an adenoma, with functional hypoplasia of the contralateral gland. On the other hand an absence of fixation in the presence of a certain Cushing syndrome would point to malignant cortical carcinoma [fr

  12. Adrenal Hypoplasia Congenita: A Rare Cause of Primary Adrenal Insufficiency and Hypogonadotropic Hypogonadism.

    Science.gov (United States)

    Loureiro, Marta; Reis, Filipa; Robalo, Brígida; Pereira, Carla; Sampaio, Lurdes

    2015-09-28

    Primary adrenal insufficiency is defined by the impaired synthesis of adrenocortical hormones due to an intrinsic disease of the adrenal cortex. Determining its etiology is crucial to allow adequate long-term management and genetic counseling. We report the case of a male adolescent that presented in the neonatal period with adrenal crisis and received replacement therapy for primary adrenal insufficiency. During follow-up, adrenal hypoplasia congenita (AHC) was suspected given his persistently raised adrenocorticotropic hormone levels, with markedly low 17-OH progesterone and androstenedione levels. DNA sequence analysis revealed a mutation in NR0B1 gene (c.1292delG), confirming the diagnosis. Delayed puberty and persistent low levels of gonadotropins led to testosterone replacement therapy. X-linked AHC is a rare cause of primary adrenal insufficiency and hypogonadotropic hypogonadism, related to mutations in NR0B1 gene. Despite its rarity, AHC should be considered in patients who present with primary adrenal failure, low levels of 17-OH progesterone and hypogonadotropic hypogonadism.

  13. Adrenal hypoplasia congenita: a rare cause of primary adrenal insufficiency and hypogonadotropic hypogonadism

    Directory of Open Access Journals (Sweden)

    Marta Loureiro

    2015-09-01

    Full Text Available Primary adrenal insufficiency is defined by the impaired synthesis of adrenocortical hormones due to an intrinsic disease of the adrenal cortex. Determining its etiology is crucial to allow adequate long-term management and genetic counseling. We report the case of a male adolescent that presented in the neonatal period with adrenal crisis and received replacement therapy for primary adrenal insufficiency. During follow-up, adrenal hypoplasia congenita (AHC was suspected given his persistently raised adrenocorticotropic hormone levels, with markedly low 17-OH progesterone and androstenedione levels. DNA sequence analysis revealed a mutation in NR0B1 gene (c.1292delG, confirming the diagnosis. Delayed puberty and persistent low levels of gonadotropins led to testosterone replacement therapy. X-linked AHC is a rare cause of primary adrenal insufficiency and hypogonadotropic hypogonadism, related to mutations in NR0B1 gene. Despite its rarity, AHC should be considered in patients who present with primary adrenal failure, low levels of 17-OH progesterone and hypogonadotropic hypogonadism.

  14. Factors predicting the duration of adrenal insufficiency in patients successfully treated for Cushing disease and nonmalignant primary adrenal Cushing syndrome.

    Science.gov (United States)

    Prete, Alessandro; Paragliola, Rosa Maria; Bottiglieri, Filomena; Rota, Carlo Antonio; Pontecorvi, Alfredo; Salvatori, Roberto; Corsello, Salvatore Maria

    2017-03-01

    Successful treatment of Cushing syndrome causes transient or permanent adrenal insufficiency deriving from endogenous hypercortisolism-induced hypothalamus-pituitary-adrenal-axis suppression. We analyzed pre-treatment factors potentially affecting the duration of adrenal insufficiency. We conducted a retrospective analysis on patients successfully treated for Cushing disease (15 patients) who underwent transsphenoidal surgery, and nonmalignant primary adrenal Cushing syndrome (31 patients) who underwent unilateral adrenalectomy, divided into patients with overt primary adrenal Cushing syndrome (14 patients) and subclinical primary adrenal Cushing syndrome (17 patients). Epidemiological data, medical history, and hormonal parameters depending on the etiology of hypercortisolism were collected and compared to the duration of adrenal insufficiency. The median duration of follow-up after surgery for Cushing disease and primary adrenal Cushing syndrome was 70 and 48 months, respectively. In the Cushing disease group, the median duration of adrenal insufficiency after transsphenoidal surgery was 15 months: younger age at diagnosis and longer duration of signs and symptoms of hypercortisolism before diagnosis and surgery were associated with longer duration of adrenal insufficiency. The median duration of adrenal insufficiency was 6 months for subclinical primary adrenal Cushing syndrome and 18.5 months for overt primary adrenal Cushing syndrome. The biochemical severity of hypercortisolism, the grade of hypothalamus-pituitary-adrenal-axis suppression, and treatment with ketoconazole before surgery accounted for longer duration of adrenal insufficiency. In patients with Cushing disease, younger age and delayed diagnosis and treatment predict longer need for glucocorticoid replacement therapy after successful transsphenoidal surgery. In patients with primary adrenal Cushing syndrome, the severity of hypercortisolism plays a primary role in influencing the duration of

  15. How Do Health Care Providers Diagnose Adrenal Gland Disorders?

    Science.gov (United States)

    ... Share Facebook Twitter Pinterest Email Print How do health care providers diagnose adrenal gland disorders? Methods for diagnosing ... Tumors To diagnose an adrenal gland tumor, a health care provider may order one or more tests. 3 ...

  16. REVIEW ARTICLE Adrenal lesions encountered in current medical ...

    African Journals Online (AJOL)

    6]. C. Positron-emission tomography. Positron-emission tomography (PET) plays an increasingly pivotal role in functional imaging, governed by the metabolic activity of the adrenal lesion. Like most other non- benign lesions, malignant adrenal ...

  17. What Are the Treatments for Adrenal Gland Disorders?

    Science.gov (United States)

    ... National Institute of Diabetes and Digestive and Kidney Diseases. (2012). Cushing's syndrome. Retrieved May 26, 2016, from http://www. ... Adrenal Diseases Foundation. (n.d.). Adrenal diseases-Addison's disease: The ...

  18. What Are Some Types of Adrenal Gland Disorders?

    Science.gov (United States)

    ... Tumor of the pituitary gland (this is called Cushing's disease) Tumor of the adrenal gland (as explained above) ... the adrenal glands to produce too much cortisol. Cushing's disease refers to pituitary tumors that cause Cushing's syndrome . ...

  19. Magnetic resonance appearance of adrenal hemorrhage in a neonate

    International Nuclear Information System (INIS)

    Willemse, A.P.P.; Feldberg, M.A.M.; Witkamp, T.D.; Coppes, M.J.; Kramer, P.P.G.

    1989-01-01

    The Magnetic Resonance (MR) appearance of adrenal hemorrhage in a neonate is described and compared with Ultrasound (US). The value of US studies in adrenal neonatal hemorrhage is well known. We present the MR appearance of this common condition. (orig.)

  20. Neurologic complications of disorders of the adrenal glands.

    Science.gov (United States)

    Bertorini, Tulio E; Perez, Angel

    2014-01-01

    Disorders of the adrenal glands frequently have secondary neurological manifestations, while some diseases that involve the central nervous system are accompanied by adrenal gland dysfunction. Excessive corticosteroid secretions in primary or secondary Cushing's syndrome causes muscle weakness and behavioral disturbances, such as emotional lability and sometimes depression, while adrenal insufficiency may cause fatigue, weakness, and depression. Adrenoleukodystrophy and adrenoneuromyelopathy are X-linked recessive disorders of the metabolism of very long chain fatty acids that manifest with white matter abnormalities of the brain, myelopathy and/or neuropathy, as well as adrenal insufficiency. Other disorders of the adrenal glands include hyperaldosteroidism, which may cause weakness from hypokalemia. Dysfunction of the adrenal medulla causes excessive or deficient secretion of catecholamines, primarily causing cardiovascular symptoms. This chapter reviews the clinical manifestations and diagnostic aspects and treatment of the various disorders of the adrenal glands. Some of the congenital adrenal diseases are also discussed. © 2014 Elsevier B.V. All rights reserved.

  1. [Combined modality therapy for a patient with primary adrenal lymphoma].

    Science.gov (United States)

    Matsuno, Teppei; Kuroda, Hiroyuki; Jomen, Wataru; Yoshida, Masahiro; Yamada, Michiko; Sato, Masanori; Abe, Tomoyuki; Sakurai, Tamaki; Fujii, Shigeyuki; Maeda, Masahiro; Fujita, Miri; Nagashima, Kazuo; Nojiri, Shuichi; Arihara, Yohei; Kato, Junji

    2014-04-01

    A 71-year-old man with malaise, anorexia, and weight loss was referred to our hospital from a clinic. Abdominal computed tomography(CT)revealed bilateral adrenal masses. An ultrasound-guided percutaneous needle biopsy of the adrenal grand indicated diffuse large B-cell lymphoma. A rapid adrenocorticotropic hormone(ACTH)test revealed primary adrenal failure. Rituximab-cyclophosphamide/doxorubicin/vincristine/prednisolone(common name, R-CHOP)therapy accompanied by intrathecal treatment was initiated along with steroid replacement therapy. After the fourth courses, a CT scan showed a reduction of the adrenal masses, and there was no[18F]-fluorodeoxyglucose(FDG)uptake in the adrenal masses. The patient has remained in metabolic complete remission. Subsequently, both adrenal lymphomas were irradiated. The patient has been disease-free for 6 months after the diagnosis of primary adrenal lymphoma. The combined modality of chemoradiation therapy plus intrathecal treatment could be effective for primary adrenal lymphoma with a poor prognosis.

  2. Clinical significance of adrenal computed tomography in Addison's disease

    International Nuclear Information System (INIS)

    Sun, Zhong-Hua; Nomura, Kaoru; Toraya, Shohzoh; Ujihara, Makoto; Horiba, Nobuo; Suda, Toshihiro; Tsushima, Toshio; Demura, Hiroshi; Kono, Atsushi

    1992-01-01

    Adrenal computed tomographic (CT) scanning was conducted in twelve patients with Addison's disease during the clinical course. In tuberculous Addison's disease (n=8), three of four patients examined during the first two years after disease onset had bilaterally enlarged adrenals, while one of four had a unilaterally enlarged one. At least one adrenal gland was enlarged after onset in all six patients examined during the first four years. Thereafter, the adrenal glands was atrophied bilaterally, in contrast to adrenal glands in idiopathic Addison's disease which was atrophied bilaterally from disease onset (n=2). Adrenal calcification was a less sensitive clue in tracing pathogenesis, i.e., adrenal calcification was observed in five of eight patients with tuberculous Addison's disease, but not idiopathic patients. Thus, adrenal CT scanning could show the etiology of Addison's disease (infection or autoimmunity) and the phase of Addison's disease secondary to tuberculosis, which may be clinically important for initiating antituberculous treatment. (author)

  3. Imaging of the adrenal gland lesions

    Directory of Open Access Journals (Sweden)

    Keith Herr

    2014-08-01

    Full Text Available With the steep increase in the use of cross-sectional imaging in recent years, the incidentally detected adrenal lesion, or "incidentaloma", has become an increasingly common diagnostic problem for the radiologist, and a need for an approach to classifying these lesions as benign, malignant or indeterminate with imaging has spurred an explosion of research. While most incidentalomas represent benign disease, typically an adenoma, the possibility of malignant involvement of the adrenal gland necessitates a reliance on imaging to inform management decisions. In this article, we review the literature on adrenal gland imaging, with particular emphasis on computed tomography, magnetic resonance imaging, and photon-emission tomography, and discuss how these findings relate to clinical practice. Emerging technologies, such as contrast-enhanced ultrasonography, dual-energy computed tomography, and magnetic resonance spectroscopic imaging will also be briefly addressed.

  4. Principles and management of adrenal cancer

    International Nuclear Information System (INIS)

    Javadpour, N.

    1987-01-01

    This book provides information on adrenal diseases of latest developments and guides the clinicians in the care of their patients. The book is divided into two parts. The first section gives an overview of the embryology, anatomy, physiology, markers, pathology, imaging and the current progress in the field. The second edition covers specific diseases of the adrenal cortex and medulla. The increasingly significant roles played by steroids, catecholamines, blockers, computed tomography and magnetic resonance are elucidated and discussed. The contents include: Overview of progress; current problems, and perspectives - embryology anatomy, physiology, and biologic markers; pathology; advances in diagnosis; imaging techniques; adrenal disorders in childhood; primary aldosteronism; Cushing's syndrome; carcinoma; pheochromocytoma; neuroblastoma; metastatic disease; surgical management; and subject index

  5. Principles and management of adrenal cancer

    Energy Technology Data Exchange (ETDEWEB)

    Javadpour, N.

    1987-01-01

    This book provides information on adrenal diseases of latest developments and guides the clinicians in the care of their patients. The book is divided into two parts. The first section gives an overview of the embryology, anatomy, physiology, markers, pathology, imaging and the current progress in the field. The second edition covers specific diseases of the adrenal cortex and medulla. The increasingly significant roles played by steroids, catecholamines, blockers, computed tomography and magnetic resonance are elucidated and discussed. The contents include: Overview of progress; current problems, and perspectives - embryology anatomy, physiology, and biologic markers; pathology; advances in diagnosis; imaging techniques; adrenal disorders in childhood; primary aldosteronism; Cushing's syndrome; carcinoma; pheochromocytoma; neuroblastoma; metastatic disease; surgical management; and subject index.

  6. Imaging of the adrenal gland lesions

    Energy Technology Data Exchange (ETDEWEB)

    Herr, Keith [Department of Radiology, Keck School of Medicine, University of Southern California, Los Angeles, CA (United States); Muglia, Valdair F. [Universidade de Sao Paulo (FMRP/USP), Ribeirao Preto, SP (Brazil). Faculdade de Medicina; Koff, Walter Jose [Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, RS (Brazil). Faculdade de Medicina. Dept. de Cirurgia; Westphalen, Antonio Carlos, E-mail: antonio.westphalen@ucsf.edu [Departments of Radiology and Biomedical Imaging and Urology, School of Medicine, University of California, San Francisco, CA (United States)

    2014-07-15

    With the steep increase in the use of cross-sectional imaging in recent years, the incidentally detected adrenal lesion, or 'incidentaloma', has become an increasingly common diagnostic problem for the radiologist, and a need for an approach to classifying these lesions as benign, malignant or indeterminate with imaging has spurred an explosion of research. While most incidentalomas represent benign disease, typically an adenoma, the possibility of m alignant involvement of the adrenal gland necessitates a reliance on imaging to inform management decisions. In this article, we review the literature on adrenal gland imaging, with particular emphasis on computed tomography, magnetic resonance imaging, and photon-emission tomography, and discuss how these findings relate to clinical practice. Emerging technologies, such as contrast-enhanced ultrasonography, dual-energy computed tomography, and magnetic resonance spectroscopic imaging will also be briefly addressed. (author)

  7. Adrenal insufficiency in pakistani hiv infected patients

    International Nuclear Information System (INIS)

    Afreen, B.; Khan, K.A.; Riaz, A.

    2017-01-01

    Background: Adrenal insufficiency (AI) is the most common endocrine complication among patients with AIDS/HIV infection and there are number of causes of AI in HIV patients. Human immunodeficiency virus directly as well as indirectly destroys adrenal glands. The estimates of its prevalence and severity vary. AI is the most life threatening but readily correctable endocrine complication that occurs in persons with HIV infection. This study was carried out to determine the frequency of Adrenal Insufficiency in HIV patients and their clinical features as proper diagnosis and timely treatment have been shown to improve quality of life and long-term mortality in AIDS patients. Methods: It was a cross sectional survey conducted at HIV clinic and Jinnah Allama Iqbal Institute of Diabetes and Endocrinology, Jinnah Hospital Lahore. Sixty-four HIV positive patients, both male and female, aged above 15 years were included in the study. HIV patients who had recently taken steroids, ketoconazole or rifampicin, determined on history, were excluded from the study. The data was collected on a structured proforma and analysis was performed in SPSS-21.0. Frequency and percentages for adrenal insufficiency and its characteristics were calculated. Chi-square test was used with p<0.05 as statistically significant. Results: In this study, 9 (14.06%) HIV patients were diagnosed with adrenal insufficiency, male to female ratio was 3.5:1 and AI was found statistically significantly associated with fatigue (p<0.008) and weight loss (p<0.001). Conclusion: Adrenal insufficiency was high among the patients with HIV, it was not gender specific but it was found to be associated with fatigue and weight loss. (author)

  8. Ovarian adrenal interactions during the menopausal transition.

    Science.gov (United States)

    Lasley, B L; Crawford, S L; McConnell, D S

    2013-12-01

    Observations over the past decade using longitudinal data reveal a gender-specific shift in adrenal steroid production. This shift is represented by an increase in the circulating concentrations of delta 5 steroids in 85% of all women and is initiated only after the menopausal transition has begun. While the associated rise in the major adrenal androgen, dehydroepiandrosterone sulfate (DHEAS), is modest, the parallel rises in dehydroepiandrosteone (DHEA) and androstenediol (Adiol) are much more robust. These increases in circulating steroid concentrations are qualitatively similar on average between ethnicities but quantitatively different between individual women. Both circulating testosterone (T) and androstenedione (Adione) also rise concomitantly but modestly by comparison. This phenomenon presents a new and provocative aspect to the endocrine foundations of the menopausal transition and may provide important clues to understanding the fundamentals of mid-aged women's healthy aging, particularly an explanation for the wide diversity in phenotypes observed during the MT as well as their different responses to hormone replacement therapies. Experimental studies using the nonhuman primate animal model show an acute adrenal response to human chorionic gonadotropin (hCG) challenge as well as the presence of luteinizing hormone receptors (LHR) in their adrenal cortices. These experimental results support the concept that LHRs are recruited to the adrenal cortices of mid-aged women that subsequently function to respond to increasing circulating LH to shunt pregnenolone metabolites towards the delta 5 pathway. Future investigations are required to determine the relationship of these changes in adrenal function to symptoms and health outcomes of mid-aged women.

  9. Bilateral adrenal hemorrhage and primary antiphospholipid antibody syndrome

    International Nuclear Information System (INIS)

    Garcia de Iturrospe, C.; Quilez, I.J.; Echevarria, J.J.

    1996-01-01

    Bilateral adrenal hemorrhage is an uncommon entity that is difficult to diagnose given the ambiquity of the clinical signs. Computerized tomography plays a major role in the diagnosis, disclosing enlarged adrenal glands presenting the hyperdense aspect that characterizes this disorders. We present a case of bilateral adrenal hemoorrhage in a patient diagnosed as having primary antiphospholipid antibody syndrome, which is a less common cause of adrenal hemorrhage than those classically reported, such as anticoagulant therapy, sepsis, shock and abdominal injury. (Author) 8 refs

  10. Two cases of neonatal adrenal hemorrhage presenting with persistent jaundice.

    Science.gov (United States)

    Ruffini, E; De Petris, L; Zorzi, G; Paoletti, P; Mambelli, G; Carlucci, A

    2013-01-01

    The adrenal hemorrhage is a relatively rare event in newborns but must be considered in the presence of a persistent unexplained jaundice, especially in presence of predisposing factors. Serial ultrasonography is the modality of choice for initial diagnosis and follow-up of neonatal adrenal hemorrhage. We report two cases of neonatal adrenal hemorrhage presenting with persistent jaundice. The causes of the neonatal adrenal hemorrhages were a difficult vaginal delivery in macrosomic infant and a neonatal infection.

  11. Adult adrenal haemorrhage: an unrecognised complication of renal vein thrombosis

    Energy Technology Data Exchange (ETDEWEB)

    Loke, T.K.L. E-mail: lokekl@ha.org.hk

    2001-07-01

    There are many predisposing factors for neonatal adrenal haemorrhage but the causative factors are different in adults. Several cases of neonatal adrenal haemorrhage have been reported in association with renal vein thrombosis. This complication has not been documented in the adults. The presence of an adrenal mass in the setting of renal vein thrombosis should raise the possibility of adrenal haemorrhage even though this is extremely uncommon in adults.

  12. Adrenal glands in patients with cogenital renal anomalies: CT appearance

    Energy Technology Data Exchange (ETDEWEB)

    Kenney, P.J.; Robbins, G.L.; Ellis, D.A.; Spirt, B.A.

    1985-04-01

    The CT appearance of the adrenal glands was investigated in 30 patients with congenital renal anomalies. The ipsilateral adrenal was clearly identified in 83% of these patients; in all of them, the adrenal was a paraspinal disk-shaped organ, which appeared linear on CT. Conversely, the adrenals retained their normal shape in a control group of 20 patients with acquired renal atrophy or prior simple nephrectomy.

  13. An extensive denture‑induced hyperplasia of maxilla | Veena ...

    African Journals Online (AJOL)

    Elimination of the inflammation and excision of the lesion is the treatment of choice. Denture induced hyperplasia in right maxillary buccal vestibule in a middle age old female patient was presented. Surgical excision was done and new denture was fabricated. Keywords: Denture hyperplasia, Denture, Epulis fissuratum ...

  14. Amlodipine-induced gingival hyperplasia in chronic renal failure: a ...

    African Journals Online (AJOL)

    Amlodipine is a dihydropyridine calcium channel blocker that is used in the management of both hypertension and angina. Amlodipine induced side effects are headache, dizziness, edema, flushing, palpitations, and rarely gingival hyperplasia. The exact reason of amlodipine-induced gingival hyperplasia is not known.

  15. Case Report: Giant Benign Prostatic Hyperplasia in a Ghanaian ...

    African Journals Online (AJOL)

    Giant Benign Prostatic Hyperplasia (GBPH) is an uncommon pathology of the prostate gland. Up to date, only 17 cases have been described with specimen weights exceeding 500 g in the world literature. We report the successful removal of the largest ever benign prostatic hyperplasia (800 g) via transvesical prostatectomy ...

  16. Cyclooxygenase 2 promotes parathyroid hyperplasia in ESRD.

    Science.gov (United States)

    Zhang, Qian; Qiu, Junsi; Li, Haiming; Lu, Yanwen; Wang, Xiaoyun; Yang, Junwei; Wang, Shaoqing; Zhang, Liyin; Gu, Yong; Hao, Chuan-Ming; Chen, Jing

    2011-04-01

    Hyperplasia of the PTG underlies the secondary hyperparathyroidism (SHPT) observed in CKD, but the mechanism underlying this hyperplasia is incompletely understood. Because aberrant cyclooxygenase 2 (COX2) expression promotes epithelial cell proliferation, we examined the effects of COX2 on the parathyroid gland in uremia. In patients with ESRD who underwent parathyroidectomy, clusters of cells within the parathyroid glands had increased COX2 expression. Some COX2-positive cells exhibited two nuclei, consistent with proliferation. Furthermore, nearly 78% of COX2-positive cells expressed proliferating cell nuclear antigen (PCNA). In the 5/6-nephrectomy rat model, rats fed a high-phosphate diet had significantly higher serum PTH levels and larger parathyroid glands than sham-operated rats. Compared with controls, the parathyroid glands of uremic rats exhibited more PCNA-positive cells and greater COX2 expression in the chief cells. Treatment with COX2 inhibitor celecoxib significantly reduced PCNA expression, attenuated serum PTH levels, and reduced the size of the glands. In conclusion, COX2 promotes the pathogenesis of hyperparathyroidism in ESRD, suggesting that inhibiting the COX2 pathway could be a potential therapeutic target. Copyright © 2011 by the American Society of Nephrology

  17. Adrenal insufficiency in critically ill septic patients at Dr George ...

    African Journals Online (AJOL)

    Adele

    Measurements and main results: A random plasma cortisol level was measured in consecutive patients with severe sepsis and septic shock. Adrenal insufficiency was defined as a cortisol level below 20µg/dL. The incidence of adrenal insufficiency was 26, 97% (CI: 19, 97% - 34, 03%). Patients with adrenal insufficiency ...

  18. Leiomyoma: A rare cause of adrenal incidentaloma | Alteer | Journal ...

    African Journals Online (AJOL)

    Most of the reported cases of adrenal leiomyomas in the literature are of patients with HIV and/or latent Epstein-Barr virus infections. This case illustrates that benign tumours, such as leiomyomas, can mimic the imaging phenotype of adrenal cortical carcinomas, and should be included in the differential diagnosis of adrenal ...

  19. Higher-order sub-Poissonian-like nonclassical fields: Theoretical and experimental comparison

    Czech Academy of Sciences Publication Activity Database

    Peřina Jr., J.; Michálek, Václav; Haderka, O.

    2017-01-01

    Roč. 96, č. 3 (2017), s. 1-7, č. článku 033852. ISSN 2469-9926 Institutional support: RVO:68378271 Keywords : twin beams * photon statistics * sub-Poissonian-like nonclassical fields Subject RIV: BH - Optics, Masers, Lasers OBOR OECD: Optics (including laser optics and quantum optics) Impact factor: 2.925, year: 2016

  20. Food Stamps and Food Insecurity: What Can Be Learned in the Presence of Nonclassical Measurement Error?

    Science.gov (United States)

    Gundersen, Craig; Kreider, Brent

    2008-01-01

    Policymakers have been puzzled to observe that food stamp households appear more likely to be food insecure than observationally similar eligible nonparticipating households. We reexamine this issue allowing for nonclassical reporting errors in food stamp participation and food insecurity. Extending the literature on partially identified…

  1. The prognostic role of classical and nonclassical MHC class I expression in endometrial cancer

    NARCIS (Netherlands)

    Bijen, C.B.; Bantema-Joppe, E.J.; de Jong, Renske; Leffers, N.; Mourits, M.J.; Eggink, Henk F.; van der Zee, A.G.; Hollema, H.; de Bock, G.H.; Nijman, H.W.

    2010-01-01

    The aim of this study was to investigate classical MHC class I and nonclassical MHC (human leukocyte antigen-G [HLA-GJ) expression in a large cohort of patients with endometrial cancer, to determine the prognostic value of these cell surface markers and their relation with clinicopathological

  2. Experimental detection of nonclassicality of single-mode fields via intensity moments

    Czech Academy of Sciences Publication Activity Database

    Arkhipov, Ie.I.; Peřina, Jan; Haderka, O.; Michálek, Václav

    2016-01-01

    Roč. 24, č. 26 (2016), s. 29496-29505 ISSN 1094-4087 R&D Projects: GA ČR GAP205/12/0382 Institutional support: RVO:68378271 Keywords : experimental detection of nonclassicality * single-mode fields * intensity moments Subject RIV: BH - Optics , Masers, Lasers Impact factor: 3.307, year: 2016

  3. Squeezing effects applied in nonclassical superposition states for quantum nanoelectronic circuits

    Science.gov (United States)

    Choi, Jeong Ryeol

    2017-06-01

    Quantum characteristics of a driven series RLC nanoelectronic circuit whose capacitance varies with time are studied using an invariant operator method together with a unitary transformation approach. In particular, squeezing effects and nonclassical properties of a superposition state composed of two displaced squeezed number states of equal amplitude, but 180° out of phase, are investigated in detail. We applied our developments to a solvable specific case obtained from a suitable choice of time-dependent parameters. The pattern of mechanical oscillation of the amount of charges stored in the capacitor, which are initially displaced, has exhibited more or less distortion due to the influence of the time-varying parameters of the system. We have analyzed squeezing effects of the system from diverse different angles and such effects are illustrated for better understanding. It has been confirmed that the degree of squeezing is not constant, but varies with time depending on specific situations. We have found that quantum interference occurs whenever the two components of the superposition meet together during the time evolution of the probability density. This outcome signifies the appearance of nonclassical features of the system. Nonclassicality of dynamical systems can be a potential resource necessary for realizing quantum information technique. Indeed, such nonclassical features of superposition states are expected to play a key role in upcoming information science which has attracted renewed attention recently.

  4. "Nonclassical" secretion of annexin A2 to the lumenal side of the enterocyte brush border membrane

    DEFF Research Database (Denmark)

    Danielsen, E Michael; van Deurs, Bo; Hansen, Gert H

    2003-01-01

    side of the microvilli, showing an apical secretion by a "nonclassical" mechanism. In addition, annexin A2 was associated with surface-connected, deep apical tubules in the apical terminal web region and with an underlying pleiomorphic, tubulo-vesicular compartment (subapical compartment...

  5. Nanoparticle-mediated nonclassical crystal growth of sodium fluorosilicate nanowires and nanoplates

    Directory of Open Access Journals (Sweden)

    Hongxia Li

    2011-12-01

    Full Text Available We observed nonclassical crystal growth of the sodium fluorosilicate nanowires, nanoplates, and hierarchical structures through self-assembly and aggregation of primary intermediate nanoparticles. Unlike traditional ion-by-ion crystallization, the primary nanoparticles formed first and their subsequent self-assembly, fusion, and crystallization generated various final crystals. These findings offer direct evidences for the aggregation-based crystallization mechanism.

  6. Feature-based prediction of non-classical and leaderless protein secretion

    DEFF Research Database (Denmark)

    Bendtsen, Jannick Dyrløv; Jensen, Lars Juhl; Blom, Nikolaj

    2004-01-01

    -containing) secretory proteins where only the mature part of the protein has been annotated or cases where the signal peptide remains uncleaved. By scanning the entire human proteome we identified new proteins potentially undergoing non-classical secretion. Predictions can be made at http://www.cbs.dtu.dk/services/SecretomeP....

  7. Systematic review and metaanalysis on nonclassic cardiovascular biomarkers after hypertensive pregnancy disorders

    NARCIS (Netherlands)

    Visser, Sanne; Hermes, Wietske; Ket, Johannes C. F.; Otten, René H. J.; van Pampus, Maria G.; Bloemenkamp, Kitty W. M.; Franx, Arie; Mol, Ben W.; de Groot, Christianne J. M.

    2014-01-01

    The aim of this study was to investigate which nonclassic cardiovascular biomarkers are associated with persistent endothelial dysfunction after pregnancy in women with a history of hypertensive pregnancy disorders compared with women with uncomplicated pregnancies. This was a systematic review and

  8. Imaging features of benign adrenal cysts

    International Nuclear Information System (INIS)

    Sanal, Hatice Tuba; Kocaoglu, Murat; Yildirim, Duzgun; Bulakbasi, Nail; Guvenc, Inanc; Tayfun, Cem; Ucoz, Taner

    2006-01-01

    Benign adrenal gland cysts (BACs) are rare lesions with a variable histological spectrum and may mimic not only each other but also malignant ones. We aimed to review imaging features of BACs which can be helpful in distinguishing each entity and determining the subsequent appropriate management

  9. Total Body Opacification 'Technique Neonatal Adrenal Haemorrhage

    African Journals Online (AJOL)

    1971-12-11

    Dec 11, 1971 ... A case is reported illustrating the possible usefulness of total body opacification in the diagnosis of neonatal adrenal haemorrhage. To derive maximum benefit from this principle, the routine use of an early film coupled with high dosage is urged whenever an intravenous pyelogram is performed for ...

  10. Adrenal incidentaloma in neurofibromatosis type 1

    Directory of Open Access Journals (Sweden)

    Tančić-Gajić Milina

    2008-01-01

    Full Text Available INTRODUCTION Neurofibromatosis type 1 is one of the most common genetically transmitted diseases with a high index of spontaneous mutations and extremely varied and unpredictable clinical manifestations. It is diagnosed by the existence of certain clinical criteria. The presence of numerous localised cutaneous neurofibromas or a plexiform neurofibroma is virtually pathognomonic of neurofibromatosis type 1. The incidence of pheochromocytoma in neurofibromatosis type 1 is 0.1-5.7%. CASE OUTLINE A 56-year old female patient was admitted for further evaluation of incidental adrenal tumour previously diagnosed on computerized tomography (CT. She had previously unrecognized neurofibromatosis type 1 and a clinical picture which could remind of pheochromocytoma. None of the catecholamine samples in 24 hr urine indicated functionally active pheochromocytoma. Chromogranin A was moderately increased. Decision for operation was made after performing the image techniques. Adrenal incidentaloma had features of pheochromocytoma on abdominal magnetic resonance imaging (MRI, with positive 131I-MIBG (iodine 131-labelled metaiodobenzylguanidine scintigraphy. After being treated with phenoxybenzamine and propranolol, she was operated on. The pathohistological finding showed the case of left adrenal pheochromocytoma. CONCLUSION Detailed diagnostic procedure for pheochromocytoma should be performed with patients having neurofibromatosis type 1 and adrenal incidentaloma. Pheochromocytomas are rare tumours with fatal outcome if not duly recognized and cured.

  11. Adrenal metabolism of mitotane and related compounds

    International Nuclear Information System (INIS)

    Djanegara, T.K.S.

    1989-01-01

    Mitotane (o,p'-DDD; 1-[2-chlorophenyl]-1-[4-chlorophenyl]-2,2-dichloroethane) has been used in the treatment of Cushing's syndrome due to adrenal hyperfunction and it the drug of choice for adrenocortical carcinoma. The object of this investigation is to study the biotransformation of o,p'-DDD and p,p'-DDD in dogs and bovine adrenal cortex to explain its selective toxicity and mechanism of action. The in vitro biotransformation of 14 C-labeled o,p'-DDD and p,p'-DDD by dog and bovine adrenal cortex as studied. Of the cortex subcellular fractions, the cytosol fraction was found to be the most active in metabolizing the substrates, followed by the mitochondrial fraction. This metabolism including that in cytosolic fractions, did not take place with boiled enzyme preparations and required an NADPH generating system. This study has been directed towards establishing the metabolic activation mechanism which may account for the adrenocorticolytic effect of mitotane in contrast to detoxication by the liver. HPLC and TLC metabolic profiles have been generated from incubations of bovine and dog adrenal cortex homogenates and their subfractions for 14 C-labeled p,p'-DDD, o,p'-DDD and its monochloroethylene derivative, o,p'-DDMU

  12. Management of benign prostatic hyperplasia with silodosin

    Directory of Open Access Journals (Sweden)

    Tomonori Yamanishi

    2009-08-01

    Full Text Available Tomonori Yamanishi1, Tomoya Mizuno1, Takao Kamai1, Ken-ichiro Yoshida1, Ryuji Sakakibara2, Tomoyuki Uchiyama31Department of Urology, Dokkyo Medical University, Tochigi, Japan; 2Department of Neurology, Sakura Hospital, Toho University, Toho, Japan; 3Department of Neurology, Chiba University, Chiba, JapanAbstract: It has been reported that blockade of α1A-adrenoceptor (AR relieves bladder outlet obstruction, while blockade of α1D-AR is believed to alleviate storage symptoms due to detrusor overactivity. Silodosin, (--1-(3-hydroxypropyl-5-[(2R-2-({2-[2-(2,2,2trifluoroethoxy phenoxy]ethyl}aminopropyl]-2,3-dihydro-1H-indole-7- carboxamide, is a new α1A-AR selective antagonist. Silodosin is highly selective for the α1A-AR subtype, showing an affinity for the α1A-AR that is 583- and 55.5-fold higher than its affinity for the α1B- and α1D-ARs, respectively. In randomized, double-blind, placebo-controlled phase III studies performed in Japan and the United States, silodosin has been shown to be effective for both storage and voiding symptoms associated with benign prostatic hyperplasia. Early effects of silodosin (after 2–6 hours or day 1 on lower urinary tract symptoms have also been reported. In urodynamic studies, detrusor overactivity disappeared in 40% and improved in 35% of patients after administration. In pressure flow studies, the grade of obstruction on the International Continence Society nomogram showed improvement in 56% of patients. The rate of adverse events in the silodosin, tamsulosin and placebo groups was 88.6%, 82.3%, and 71.6%, respectively. The most common adverse event was (mostly mild abnormal ejaculation (28.1%. However, few patients (2.8% discontinued silodosin because of abnormal ejaculation. Orthostatic hypotension showed a similar incidence in the silodosin (2.6% and placebo (1.5% groups. In conclusion, silodosin improves detrusor overactivity and obstruction and thus may be effective for both storage and voiding

  13. Functioning adrenal myelolipoma: A rare cause of hypertension

    Directory of Open Access Journals (Sweden)

    Nagendar Jakka

    2013-01-01

    Full Text Available Co-occurrence of adrenal incidentaloma with hypertension calls for evaluation of endocrine causes including pheochromocytoma, Cushing′s disease, and primary aldosteronism. We are reporting 40-years-old man who presented with hypertension and adrenal mass. He had elevated metanephrines, histology of resected adrenal mass revealed adrenal myelolipoma, and immuno-histochemistry was positive for chromogranin A. Both his blood pressure and urinary metanephrines returned to normal after surgery. The association of hypertension and adrenal myelolipoma may not be entirely coincidental, as it may be associated with secreting catecholamine. Literature on such an uncommon association is reviewed briefly as well.

  14. [Neonatal adrenal hemorrhage revealed by jaundice: a case report].

    Science.gov (United States)

    Oulmaati, A; Hays, S; Mory-Thomas, N; Bretones, P; Bensaid, M; Jordan, I; Bonfils, M; Godbert, I; Picaud, J-C

    2012-04-01

    The clinical presentation of adrenal hemorrhage varies, depending on the extent of hemorrhage as well as the amount of adrenal cortex involved by the hemorrhage. We report here a case of neonatal adrenal hemorrhage revealed by late onset of neonatal jaundice. This adrenal hemorrhage most probably resulted from shoulder dystocia. The aim of this work was to focus on the fact that jaundice can be caused by adrenal hemorrhage and to emphasize the crucial importance of abdominal ultrasound in cases of persistent jaundice. Copyright © 2012 Elsevier Masson SAS. All rights reserved.

  15. Delay in Diagnosis of Adrenal Insufficiency Is a Frequent Cause of Adrenal Crisis

    Directory of Open Access Journals (Sweden)

    Lucyna Papierska

    2013-01-01

    Full Text Available Delay of diagnosis of primary adrenal insufficiency (PAI leads to adrenal crisis which is potentially lethal complication. The objective of our work was an assessment whether the establishment of diagnosis of adrenocortical insufficiency in Poland is so much delayed as assessed in the past. We have analysed data from 60 patients with diagnosis of PAI established in our department during the past 12 years and who are still under our care. We found that the time to diagnosis of primary adrenal insufficiency in Poland exceeds 3 months in every patient and 6 months in patients admitted with symptoms of adrenal crisis. Forty-four percent of patients were diagnosed only just after the hospitalisation due to crisis, despite the evident signs and symptoms of PAI. Lack of appetite and loss of body weight occurred in all patients and for that reason a diagnosis of chronic gastric and duodenal ulcer disease was the most often incorrect diagnosis. After the proper diagnosis and treatment, in the course of 1–11 years of observation, there was only 6 imminent adrenal crises in 5 patients. Our results indicated that training of primary care physicians in the field of recognising and treatment of adrenal insufficiency is still essential.

  16. Bilateral adrenal gland enlargement secondary to histoplasmosis mimicking adrenal metastases: diagnosis with EUS-guided FNA.

    Science.gov (United States)

    Eloubeidi, Mohamad A; Luz, Leticia P; Crowe, David R; Snowden, Cindi; Morgan, Desiree E; Arnoletti, Pablo J

    2010-05-01

    While adrenal gland histoplasmosis has been previously diagnosed by fine needle aspiration utilizing the percutaneous approach, EUS-FNA has not been employed in the diagnosis of this infection affecting both adrenal glands. We report a patient with massive bilateral adrenal enlargement due to histoplasmosis that was diagnosed by EUS-FNA. Trans-duodenal and trans-gastric fine needle aspiration biopsy of both adrenal glands was performed. Rapid onsite cytopathologic evaluation (ROSE) revealed epithelioid histiocytes, singly and in clusters consistent with granulomas. Apparent intracytoplasmic inclusions suggestive of organisms were visible. A Gomori Methenamine Silver stain (GMS) revealed abundant small intracellular budding yeasts, morphologically consistent with Histoplasma; the patient was admitted for amphotericin B intravenous infusion. His fever abated on the second day after amphotericin B was started. His urine Histoplasma antigen was positive. Fungal cultures from both adrenal EUS-FNA samples grew Histoplasma capsulatum. After a one week hospital stay, he was discharged home on itraconazole 200 mg po bid for one year. Four months after initiation of treatment, his urine Histoplasma antigen was undetectable. Nine months after initial diagnosis, the patient regained his energy level, and returned to work with complete resolution of his initial symptoms. This case highlights that EUS-FNA with ROSE can be a highly effective tool in the diagnosis of uncommon infections of the adrenal glands.

  17. Mielolipoma adrenal bilateral em um cão Bilateral adrenal myelolipoma in a dog

    Directory of Open Access Journals (Sweden)

    Silvia de Araújo França

    2008-08-01

    Full Text Available O presente relato descreve um caso de mielolipoma adrenal bilateral em uma cadela Poodle de 13 anos de idade. O cão morreu por peritonite séptica aguda, decorrente de ulceração e perfuração intestinal por corpo estranho linear. O neoplasma foi achado incidentalmente durante a necropsia. Macroscopicamente, as adrenais estavam redondas e com volume e aumentados. Ao corte elas eram friáveis, vermelho-amarronzadas, intercaladas por áreas branco-amareladas levemente proeminentes. Histologicamente, extensas áreas do parênquima continham adipócitos bem diferenciados, células hematopoéticas e macrófagos com hemossiderina, achados característicos de mielolipoma adrenal.A case of bilateral adrenal myelolipoma in a 13-year-old female Poodle is reported. Acute septic peritonitis due to intestinal ulceration and perforation caused by a linear foreign body was the cause of death. The adrenal neoplasm was an incidental necropsy finding. The adrenal glands were grossly rounded, enlarged and friable, and had red-brownish parenchyma interwoven by slightly prominent white-yellowish foci. Histologically, the findings of differentiated adipocytes in large areas of the parenchyma, in association to hematopoietic cells and macrophages with hemosiderin were consistent with the diagnosis of adrenal myelolipoma.

  18. [Etiology and pathophysiology of benign prostate hyperplasia].

    Science.gov (United States)

    Roosen, A; Gratzke, C; Herrlemann, A; Magistro, G; Strittmatter, F; Weinhold, P; Tritschler, S; Stief, C G

    2013-02-01

    The pathogenesis of benign prostate hyperplasia (BPH) is still unclear. It is a common disease affecting exclusively humans in its full clinical appearance. There is a broad variety of possible underlying mechanisms which most likely interact in the pathogenesis of the disease: inflammatory processes taking place predominantly in the stroma and inducing proliferation of all tissues within the transitional zone, an imbalance of androgens and estrogens and their receptors, hyperinsulinemia and hypercholesterolemia (metabolic syndrome) as direct promoters of glandular growth and autosomal dominant inheritance. The detrusor muscle responds to the increased outflow resistance with muscular hypertrophy. Decreased compliance of the bladder wall results in voiding difficulties while electric instability of the hypertrophied detrusor muscle and increased recruiting of otherwise silent afferent fibres cause storage symptoms.

  19. Computed tomography of the adrenals in patients with tuberculosis

    International Nuclear Information System (INIS)

    Yamashita, Seizo; Machado, Jussara Marcondes; Morceli, Jose

    2004-01-01

    We evaluated the adrenals of individuals without tuberculosis (group 1 - G1) and with tuberculosis (group 2 - G2) using computed tomography. The antero-posterior length, the thickness, and the longitudinal length of the adrenals were compared in G1 and G2. The duration of the disease, the occurrence of morphologic abnormalities in G2, the distribution according to sex, age and skin color were also studied. There was difference in the antero-posterior length and thickness of right adrenal between G1 and G2. A higher prevalence of white skin male individuals was observed in G2. There was no association between duration of the disease and the occurrence of morphologic abnormalities in G2. A higher occurrence of adrenal enlargement was observed in G2. The main computed tomography findings in patients with tuberculosis were enlargement of the adrenal without calcification, enlargement of the adrenal with punctiform calcifications, and residual calcification of the adrenal. (author)

  20. Blunt adrenal gland trauma in the pediatric population

    Directory of Open Access Journals (Sweden)

    Stylianos Roupakias

    2011-07-01

    Full Text Available A retrospective review of the literature was performed to determine the natural history, prevalence, prognosis and management of adrenal injury associated with blunt abdominal trauma in pediatric population. Blunt adrenal injury in children is uncommon, rarely isolated, and typically present as part of a multi organ trauma. Adrenal hemorrhage is being diagnosed more frequently since the emergence of computed tomography in modern emergency rooms. Obstetric birth trauma during vaginal delivery of a macrosomic fetus may result in neonatal adrenal hemorrhage. In children appear to be an incidental finding that resolves on follow-up imaging. Most of these injuries are self-limited and do not require intervention. The differential diagnosis of an adrenal neoplasm, especially in children with an isolated adrenal hemorrhage, must be considered. The presence of adrenal hemorrhage in the absence of a trauma history should alert to the possibility of pediatric inflicted injury.

  1. Adrenal insufficiency presenting as hypercalcemia and acute kidney injury.

    Science.gov (United States)

    Ahn, Seung Won; Kim, Tong Yoon; Lee, Sangmin; Jeong, Jeong Yeon; Shim, Hojoon; Han, Yu Min; Choi, Kyu Eun; Shin, Seok Joon; Yoon, Hye Eun

    2016-01-01

    Adrenal insufficiency is an uncommon cause of hypercalcemia and not easily considered as an etiology of adrenal insufficiency in clinical practice, as not all cases of adrenal insufficiency manifest as hypercalcemia. We report a case of secondary adrenal insufficiency presenting as hypercalcemia and acute kidney injury in a 66-year-old female. The patient was admitted to the emergency department with general weakness and poor oral intake. Hypercalcemia (11.5 mg/dL) and moderate renal dysfunction (serum creatinine 4.9 mg/dL) were shown in her initial laboratory findings. Studies for malignancy and hyperparathyroidism showed negative results. Basal cortisol and adrenocorticotropic hormone levels and adrenocorticotropic hormone stimulation test confirmed the diagnosis of adrenal insufficiency. With the administration of oral hydrocortisone, hypercalcemia was dramatically resolved within 3 days. This case shows that adrenal insufficiency may manifest as hypercalcemia and acute kidney injury, which implicates that adrenal insufficiency should be considered a cause of hypercalcemia in clinical practice.

  2. Nonclassicality Generated by Applying Hermite-Polynomials Photon-Added Operator on the Even/Odd Coherent States

    Science.gov (United States)

    Ren, Gang; Du, Jian-ming; Zhang, Wen-hai; Yu, Hai-jun

    2017-05-01

    We examine nonclassical properties of the quantum state generated by applying Hermite polynomials photon-added operator on the even/odd coherent state (HPECS/HPOCS). Explicit expressions for its nonclassical properties, such as quantum statistical properties and squeezing phenomenon, are obtained. It is interesting to find that the HPECS/HPOCS exhibits sub-Poissonian distribution, anti-bunching effects and negative values of the Wigner function. Thus, we confirm the HPPECS/HPPOCS is a new nonclassical state. Finally, we reveal that the HPPECS/HPPOCS is a novel intelligent state by its squeezing effects in position distribution and quadrature squeezing.

  3. Fibroadenoma with "immature-like" type of usual ductal hyperplasia.

    Science.gov (United States)

    Bezić, Joško; Karaman, Ivana; Kunac, Nenad

    2016-01-01

    We herein report a case of the breast fibroadenoma with foci of so-called immature variant of the conventional ductal hyperplasia. This type of usual ductal hyperplasia is histologically characterised by encircling intraductal proliferation of large cells with pale to amphophilic cytoplasm and large nuclei which vary in shape and in staining quality of the chromatin. We showed here, using the cytokeratin immunohistochemistry, that the proliferating cells were not of immature but rather mature immunohistochemical phenotype. Because of the presented discordance between immature histology and mature immunohistological profile we suggest that this rare type of usual ductal hyperplasia should be called "immature-like".

  4. Maxillary sinus marrow hyperplasia in sickle cell anemia

    International Nuclear Information System (INIS)

    Fernandez, M.; Slovis, T.L.; Whitten-Shurney, W.

    1995-01-01

    Marrow hyperplasia is a sequela of sickle cell anemia (SCA) and may be seen in the skull in children after 5 years of age. The facial bones, except for the mandible and orbits, are usually not involved. We report an unusual case of a 28-month-old black boy with SCA who presented with extensive marrow hyperplasia of the maxillary sinuses in addition to severe calvarial and mandibular changes. The imaging characteristics on CT (similar to other sites of marrow hyperplasia) and MR (low signal on both T 1 and T 2 sequences) should aid in making the correct diagnosis. (orig.)

  5. Mielolipoma gigante bilateral da glândula adrenal Bilateral giant adrenal myelolipoma

    Directory of Open Access Journals (Sweden)

    Gisele Alborghetti Nai

    2007-08-01

    Full Text Available Mielolipomas são tumores benignos relativamente raros, freqüentemente associados à glândula adrenal. Geralmente são não-funcionantes, assintomáticos e medem menos de 5 cm. Mielolipomas adrenais bilaterais e gigantes são raros. Relatamos o primeiro caso de mielolipoma gigante bilateral da glândula adrenal da literatura latino-americana: paciente do sexo feminino, 57 anos, com queixa de dor no flanco esquerdo. A tomografia computadorizada do abdome mostrou massas bilaterais da glândula adrenal, medindo a direita 9 x 7 x 6,5 cm e a esquerda 23 x 19 x 8 cm. As massas eram radiologicamente consistentes com tecido adiposo. O diagnóstico pré-operatório foi de lipossarcoma. A paciente foi submetida à laparotomia com retirada das massas. O exame microscópico diagnosticou mielolipomas bilaterais. Embora sejam tumores benignos, seu tratamento deve ser cirúrgico, pois somente o exame anatomopatológico pode descartar uma neoplasia maligna.Myelolipomas are relatively rare benign tumors, frequently associated with the adrenal glands. Usually, they are non-functioning, asymptomatic and measure less than 5 cm. Bilateral giant adrenal myelolipomas are rare. We report the first case of bilateral giant adrenal myelolipomas of the Latin American literature: a 57-year old woman who presented with left abdominal pain. The computed tomography scanning revealed bilateral suprarenal masses measuring 9 x 7 x 6.5 cm on the right and 23 x 19 x 8 cm on the left. The masses were radiographically consistent with fatty tissue. The preoperative diagnosis was liposarcoma. Surgery was performed with excision of the masses. The histologic analysis identified bilateral adrenal myelolipomas. Although they are benign tumors, surgery must be performed because only the histologic analysis can exclude malignancy.

  6. [Classical and non-classical taxonomy: where does the boundary pass?].

    Science.gov (United States)

    Pavlinov, I Ia

    2006-01-01

    Rise of non-classical science during XX century had certain influence upon development of biological taxonomy. Scientific pluralism (especially normative naturalism of Laudan), contrary to positivist and early post-positivist treatments, made taxonomy acknowledged scientific discipline of its own right. The present state of some schools of taxonomy makes it possible to consider them as a part of non-classical science and constituting the non-classical taxonomy. The latter is characterized by the following most important features. Ontological substantiation of both classificatory approaches and particular classifications is requested which invalidates such formal approaches as nominalistic and phenetic (numerical) schools. This substantiation takes a form of content-wise background preferably causal models which include certain axioms and presumptions about taxonomic diversity being studied, together with its causes, and thus define initial conditions of classificatory procedures. From this viewoint, phylogenetic classificatory approach is the most developed part of non-classical taxonomy. The entire taxonomic diversity is structured into several aspects of different levels of generality, each being outlined by a particular consideration aspect. The latter makes personal knowledge constituting an irremovable part of any scientific statement about taxonomic diversity, thus opposition of "objectively" and "subjectively" elaborated classifications becomes vague. Interrelation of various species concepts corresponding to its different consideration aspects is described by uncertainty relation principle. Classificatory algorithms are to be compatible with the conditions of a background model to ensure particular classifications obtained by their means are interpretable within the same model: this is provided by the correspondence principle. Classification is considered as a taxonomic hypothesis, i.e. a conjectural judgement about structure of particular fragment of

  7. Sexual Differentiation of Circadian Clock Function in the Adrenal Gland.

    Science.gov (United States)

    Kloehn, Ian; Pillai, Savin B; Officer, Laurel; Klement, Claire; Gasser, Paul J; Evans, Jennifer A

    2016-05-01

    Sex differences in glucocorticoid production are associated with increased responsiveness of the adrenal gland in females. However, the adrenal-intrinsic mechanisms that establish sexual dimorphic function remain ill defined. Glucocorticoid production is gated at the molecular level by the circadian clock, which may contribute to sexual dimorphic adrenal function. Here we examine sex differences in the adrenal gland using an optical reporter of circadian clock function. Adrenal glands were cultured from male and female Period2::Luciferase (PER2::LUC) mice to assess clock function in vitro in real time. We confirm that there is a pronounced sex difference in the intrinsic capacity to sustain PER2::LUC rhythms in vitro, with higher amplitude rhythms in adrenal glands collected from males than from females. Changes in adrenal PER2::LUC rhythms over the reproductive life span implicate T as an important factor in driving sex differences in adrenal clock function. By directly manipulating hormone levels in adult mice in vivo, we demonstrate that T increases the amplitude of PER2::LUC rhythms in adrenal glands of both male and female mice. In contrast, we find little evidence that ovarian hormones modify adrenal clock function. Lastly, we find that T in vitro can increase the amplitude of PER2::LUC rhythms in male adrenals but not female adrenals, which suggests the existence of sex differences in the mechanisms of T action in vivo. Collectively these results reveal that activational effects of T alter circadian timekeeping in the adrenal gland, which may have implications for sex differences in stress reactivity and stress-related disorders.

  8. MRI evaluation of pituitary hyperplasia due to primary hypothyroidism

    International Nuclear Information System (INIS)

    Jiang Chunjing; Shu Jin'er; Li Huimin; Sheng Sanlan; Lu Jinhua

    2010-01-01

    Objective: To analyze the MRI manifestations of the pituitary hyperplasia due to primary hypothyroidism and to improve the differential diagnosis of secondary pituitary hyperplasia and pituitary tumors. Methods: The MRI findings of pituitary hyperplasia in 10 documented primary hypothyroidism patients (male 3, female 7; age range: 9-15 years) were reviewed. The pulse sequences using a 1.0T MR scanner included coronal and sagittal T 1 W, coronal T 2 W and coronal contrast-enhanced T 1 W in all patients. Results: The pituitary gland was markedly enlarged with mean height of 15.5 mm (11-23 mm). Central bulging of pituitary gland was seen in all 10 patients with mild displacement of the infundibulum in 3 and sellar enlargement in 5. All glands had homogeneous MR signal intensities and contrast enhancement. Conclusion: Pituitary hyperplasia due to primary hypothyroidism has characteristic MR features of central bulging with homogeneous signal intensities and contrast enhancement. (authors)

  9. Subclinical hypercortisolism in patients with adrenal incidentaloma

    International Nuclear Information System (INIS)

    Fernández, B.; Betancourt, V.C.; Padilla, P.; De la Barca, M.

    2016-01-01

    Subclinical hypercortisolism (HS) is diagnosed in a patient with no Cushing's syndrome manifest phenotype but with autonomic cortisol secretion. Harmful effects of chronic subtle excess of cortisol have been demonstrated, such as hypertension (AHT), type 2 diabetes mellitus (DM2) or glucose intolerance, obesity, osteoporosis, vertebral fractures and dyslipidemia. It has been suggested that subclinical hypercortisolism may itself be a vascular risk factor. In order to identify the presence of subclinical hypercortisolism and its main clinical manifestations in patients with adrenal incidentalomas treated at the endocrinology service of the 'Arnaldo Milian Castro' Clinical Surgical Hospital, a descriptive cross-sectional investigation was carried out from 2012 to 2015 The sample of intentional type was made up of 34 patients older than 18 years and diagnosis of adrenal incidentalomas. Age, sex, toxic habits, questioning results, physical examination and the necessary diagnostic investigations were analyzed. The results showed a predominance of females, over 60 years old, with toxic habits and family and personal history of hypertension and DM2. Palpitations, low back pain, asthenia and obesity with pigmentation of the skin were the most frequent clinical manifestations. Hyperglycemia, dyslipidemia, and elevated levels of cortisol occurred in the majority of patients. Imaging diagnosis showed left lesions and less than 4.0 cm and adrenal adenoma was the most frequent histopathological diagnosis. It was demonstrated that subclinical hypercortisolism is present in an important group of patients with adrenal incidentalomas, is not as asymptomatic, and is related to the presence of vascular risk factors, and other comorbidities

  10. Adrenal Hyperandrogenism: Multidisciplinary Approach to Solving Problemss

    Directory of Open Access Journals (Sweden)

    L.A. Lutsenko

    2016-11-01

    Full Text Available Prevalence and possible consequences of the hyperandrogenic states in women of reproductive age stipulate the importance of timely differential diagnosis of hyperandrogenism. Taking into account the level of the development of radiological and laboratory methods of researches, an accurate diagnosis is possible, especially when diagnosing adrenal hyperandrogenism, which often occurs without significant clinical symptoms, but is associated with menstrual disorders, infertility, miscarriage.

  11. Classical and non-classical HLA class I aberrations in primary cervical squamous- and adenocarcinomas and paired lymph node metastases

    OpenAIRE

    Ferns, Debbie M.; Heeren, A. Marijne; Samuels, Sanne; Bleeker, Maaike C. G.; de Gruijl, Tanja D.; Kenter, Gemma G.; Jordanova, Ekaterina S.

    2016-01-01

    Background Tumors avoid destruction by cytotoxic T cells (CTL) and natural killer (NK) cells by downregulation of classical human leukocyte antigens (HLA) and overexpression of non-classical HLA. This is the first study to investigate HLA expression in relation to histology (squamous cell carcinoma (SCC) vs. adenocarcinoma (AC)), clinicopathological parameters and survival in a large cervical cancer patient cohort. Methods Classical (HLA-A and HLA-B/C)- and non-classical HLA molecules (HLA-E ...

  12. Paediatric cyclical Cushing's disease due to corticotroph cell hyperplasia.

    LENUS (Irish Health Repository)

    Noctor, E

    2015-06-01

    Cushing\\'s disease is very rare in the paediatric population. Although uncommon, corticotroph hyperplasia causing Cushing\\'s syndrome has been described in the adult population, but appears to be extremely rare in children. Likewise, cyclical cortisol hypersecretion, while accounting for 15 % of adult cases of Cushing\\'s disease, has only rarely been described in the paediatric population. Here, we describe a very rare case of a 13-year old boy with cyclical cortisol hypersecretion secondary to corticotroph cell hyperplasia.

  13. Proteus syndrome: MRI characteristics of plantar cerebriform hyperplasia

    International Nuclear Information System (INIS)

    Vanhoenacker, F.M.; Beuckeleer, L.H. de; Schepper, A.M. de; Deprettere, A.; Moor, A. de

    2000-01-01

    Proteus syndrome is a rare congenital hamartomatous syndrome with a variety of abnormalities. It shares many features with other congenital hamartomatous disorders, but cerebriform hyperplasia of the soles and the palms is known as a quite distinctive characteristic in the dermatologic literature. The purpose of this case report is to demonstrate the MRI features of plantar cerebriform hyperplasia in a 9-year-old boy with known Proteus syndrome. (orig.)

  14. Gonadal development and growth in 46,XX and 46,XY individuals with P450scc deficiency (congenital lipoid adrenal hyperplasia)

    DEFF Research Database (Denmark)

    Müller, J; Torsson, A; Damkjaer Nielsen, M

    1991-01-01

    pathology. Gonadectomy was performed in the 2 surviving 46,XY individuals at the age of 7 years, and histological examination showed normal testicular morphology but very few germ cells. Postmortem examination of the testes of the 2-month-old subject showed normal testicular histology, and quantitative...... normal breast and pubic hair development after oral estrogen replacement and topical testosterone administration. The glucocorticoid and mineralocorticoid replacement was adjusted in accordance with repeated measurements of serum sodium and serum potassium, plasma renin concentration and blood pressure...

  15. Pharmacogenetics of glucocorticoid replacement could optimize the treatment of congenital adrenal hyperplasia due to 21-hydroxylase deficiency

    Directory of Open Access Journals (Sweden)

    Ricardo P. P. Moreira

    2011-01-01

    Full Text Available INTRODUCTION: 21-hydroxylase deficiency is an autosomal recessive disorder that causes glucocorticoid deficiency and increased androgen production. Treatment is based on glucocorticoid replacement; however, interindividual variability in the glucocorticoid dose required to achieve adequate hormonal control has been observed. OBJECTIVE: The present study aimed to evaluate the association between polymorphic variants involved inglucocorticoid action and/or metabolism and the mean daily glucocorticoid dose in 21-hydroxylase deficiency patients. METHODS: We evaluated 53 patients with classical forms of 21-hydroxylase deficiency who were receiving cortisone acetate. All patients were between four and six years of age and had normal androgen levels. RESULTS: The P450 oxidoreductase A503V, HSD11B1 rs12086634, and CYP3A7*1C variants were found in 19%, 11.3% and 3.8% of the patients, respectively. The mean ± SD glucocorticoid dose in patients with the CYP3A7*1C and wild-type alleles was 13.9 ± 0.8 and 19.5 ± 3.2 mg/m²/d, respectively. We did not identify an association between the P450 oxidoreductase or HSD11B1 allelic variants and the mean glucocorticoid dose. CONCLUSION: Patients carrying the CYP3A7*1C variant required a significantly lower mean glucocorticoid dose. Indeed, the CYP3A7*1C allele accounted for 20% of the variability in the cortisone acetate dose. The analysis of genes involved in glucocorticoid metabolism may be useful in the optimization of treatment of 21-hydroxylase deficiency.

  16. Linking Prenatal Androgens to Gender-Related Attitudes, Identity, and Activities : Evidence From Girls With Congenital Adrenal Hyperplasia

    NARCIS (Netherlands)

    Endendijk, Joyce J.|info:eu-repo/dai/nl/330190865; Beltz, Adriene M.; McHale, Susan M.; Bryk, Kristina; Berenbaum, Sheri A.

    2016-01-01

    Key questions for developmentalists concern the origins of gender attitudes and their implications for behavior. We examined whether prenatal androgen exposure was related to gender attitudes, and whether and how the links between attitudes and gendered activity interest and participation were

  17. A Newborn with Genital Ambiguity, 45,X/46,XY Mosaicism, a Jumping Chromosome Y, and Congenital Adrenal Hyperplasia

    Directory of Open Access Journals (Sweden)

    Lei Zhang

    2013-01-01

    Full Text Available Disorders of sex development (DSD, formerly termed “intersex” conditions, arise from numerous causes. CAH secondary to 21-hydroxylase deficiency is the most common cause of DSD. Sex chromosome disorders, including sex chromosome mosaicism, are the second most common cause of DSD. We discuss a medically complex neonate with DSD presenting with ambiguous genitalia. Hormone levels suggested 21-hydroxylase deficiency. Molecular analysis revealed compound heterozygous mutations in the 21-hydroxylase gene (CYP21A2, confirming the diagnosis of CAH. Chromosome analysis revealed sex chromosome mosaicism with three cell lines: 45,X[8]/45,X,tas(Y;16(p11.32;p13.3[8]/45,X,t(Y;8(p11.32;p23.3[4] with the Y chromosome in telomere association with chromosomes 8p and 16p in different cell lines, a “jumping translocation.” Histologically, the right gonad had irregular, distended seminiferous tubules with hyperplastic germ cells contiguous with ovarian stroma and primordial follicles. The left gonad had scant ovarian stroma and embryonic remnants. Chromosome analyses showed mosaicism in both gonads: 45,X[17]/45,X,tas(Y;8(p11.32;p23.3[3]. This is the first case of coexisting CAH and 45,X/46,XY mosaicism reported in the English literature and the third case of a constitutional chromosome Y “jumping translocation.” Our report documents the medical and genetic complexity of children such as this one with ambiguous genitalia and discusses the need for a multidisciplinary team approach.

  18. Adrenal Hemangioma: A Case of Retroperitoneal Tumor

    Directory of Open Access Journals (Sweden)

    Genta Iwamoto

    2018-01-01

    Full Text Available Introduction. Adrenal hemangioma is a rare disease, with only some 60 cases reported previously. Due to the difficulty of the preoperative diagnosis of adrenal hemangioma, almost all of the cases were diagnosed by a histopathological analysis of surgical specimens. Case Presentation. A 52-year-old man was referred to our department for further examination of his left retroperitoneal tumor. He had received hemodialysis due to chronic renal failure resulting from membranous nephropathy. Computed tomography revealed a mass around his left hilum. Magnetic resonance imaging (MRI and positron-emission tomography (PET-CT were unable to confirm or deny malignancy, and tumor markers, including CEA and CA19-9, showed slight elevation. His tumor grew from 38 mm to 54 mm in diameter in 7 months of follow-up. We therefore planned retroperitoneal tumor resection with left nephrectomy. Histopathologically, hyperplastic small vessels with hemorrhaging and denaturation were seen. The endothelial cells showed no variants or division of the nucleus. Based on this diagnosis, no further therapy was performed. He has had no recurrence in the eight months since the surgery. Conclusion. We herein report a rare case of adrenal hemangioma.

  19. Genitourinary MR: Kidneys and adrenal glands

    International Nuclear Information System (INIS)

    Krestin, G.P.

    1999-01-01

    Due to its high tissue contrast and multiplanar imaging capabilites, MRI provides a detailed display of renal and adrenal anatomy. Recent technical developments overcoming the problem of respiration induced motion artifacts and the use of paramagnetic contrast agents have further improved the performance of MRI which has now evolved as an alternative or complementary imaging modality to ultrasound, excretory urography and computed tomography. Dynamic contrast-enhanced studies will usually allow to detect even small enhancing solid areas within the cyst wall. Use of a fast (turbo) spoiled gradient echo sequence allows for assessment of contrast enhancement dynamics in renal and adrenal masses. For tumor staging, the multiplanar imaging capabilities of MRI are advantageous. Perinephric extent is best detected using opposed-phase GRE images resulting in an artifical accentuation of renal contours. Extension into venous structures is best diagnosed by using a GRE sequence allowing for distinction between flowing blood and tumor thrombus. Noninvasive differentiation of adrenal lesions can be performed with an unprecedented accuracy using chemical-shift imaging. (orig.)

  20. Non-classical Signature of Parametric Fluorescence and its Application in Metrology

    Directory of Open Access Journals (Sweden)

    Hamar M.

    2014-08-01

    Full Text Available The article provides a short theoretical background of what the non-classical light means. We applied the criterion for the existence of non-classical effects derived by C.T. Lee on parametric fluorescence. The criterion was originally derived for the study of two light beams with one mode per beam. We checked if the criterion is still working for two multimode beams of parametric down-conversion through numerical simulations. The theoretical results were tested by measurement of photon number statistics of twin beams emitted by nonlinear BBO crystal pumped by intense femtoseconds UV pulse. We used ICCD camera as the detector of photons in both beams. It appears that the criterion can be used for the measurement of the quantum efficiencies of the ICCD cameras.

  1. Reproducibility of Psychological Experiments as a Problem of Post-Nonclassical Science

    Directory of Open Access Journals (Sweden)

    Vachkov I.V.,

    2016-04-01

    Full Text Available A fundamental project on reproducibility carried out in the USA by Brian Nosek in 2015 (the Reproducibility Project revealed a serious methodological problem in psychology: the issue of replication of psycho- logical experiments. Reproducibility has been traditionally perceived as one of the basic principles of the scientific method. However, methodological analysis of the modern post-nonclassical stage in the development of science suggests that this might be a bit too uncompromising as applied to psychology. It seems that the very criteria of scientific research need to be reconsidered with regard to the specifics of post-nonclassical science, and, as the authors put it, as a result, reproducibility might lose its key status or even be excluded at all. The reviewed problem and the proposed ways of coping with it are of high importance to research and practice in psychology as they define the strategies for organizing, conducting and evaluating experimental research.

  2. Nonclassical phase-space trajectories for the damped harmonic quantum oscillator

    Energy Technology Data Exchange (ETDEWEB)

    Pachon, L.A. [Departamento de Fisica, Universidad Nacional de Colombia, Bogota D.C. (Colombia); Institut fuer Physik, Universitaet Augsburg, Universitaetsstrasse 1, D-86135 Augsburg (Germany); CeiBA - Complejidad, Bogota D.C. (Colombia); Ingold, G.-L., E-mail: gert.ingold@physik.uni-augsburg.de [Institut fuer Physik, Universitaet Augsburg, Universitaetsstrasse 1, D-86135 Augsburg (Germany); Dittrich, T. [Departamento de Fisica, Universidad Nacional de Colombia, Bogota D.C. (Colombia); CeiBA - Complejidad, Bogota D.C. (Colombia)

    2010-10-05

    Graphical abstract: The phase-space path-integral approach to the damped harmonic oscillator is analyzed beyond the Markovian approximation and the appearance of nonclassical trajectories is discussed. - Abstract: The phase-space path-integral approach to the damped harmonic oscillator is analyzed beyond the Markovian approximation. It is found that pairs of nonclassical trajectories contribute to the path-integral representation of the Wigner propagating function. Due to the linearity of the problem, the sum coordinate of a pair still satisfies the classical equation of motion. Furthermore, it is shown that the broadening of the Wigner propagating function of the damped oscillator arises due to the time-nonlocal interaction mediated by the heat bath.

  3. Probing the upper limit of nonclassical rotational inertia in solid helium 4.

    Science.gov (United States)

    Rittner, Ann Sophie C; Reppy, John D

    2008-10-10

    We study the effect of confinement on solid 4He's nonclassical rotational inertia fraction (NCRIF) in a torsional oscillator by constraining it to narrow annular cells of various widths. The NCRIF exhibits an observed maximum value of 20% for annuli of approximately 100 microm width. Samples constrained to porous media or to larger geometries both have smaller NCRIF, mostly below approximately 1%. In addition, we extend the blocked-annulus experiment of Kim and Chan to solid samples with large supersolid fractions. Blocking the annulus suppresses the nonclassical decoupling from 17.1% to below the limit of our detection of 0.8%. This result demonstrates the nonlocal nature of the supersolid phenomena. At 20 mK, NCRIF depends on velocity history showing a closed hysteresis loop in different thin annular cells.

  4. High energy thermotherapy in the treatment of benign prostatic hyperplasia: results of the European Benign Prostatic Hyperplasia Study Group

    NARCIS (Netherlands)

    de la Rosette, J. J.; de Wildt, M. J.; Höfner, K.; Carter, S. S.; Debruyne, F. M.; Tubaro, A.

    1996-01-01

    We documented the results of high energy transurethral microwave thermotherapy in the treatment of benign prostatic hyperplasia. We evaluated 116 patients following transurethral microwave thermotherapy according to symptom scores, transrectal ultrasound, free voiding and pressure-flow study

  5. New and superior adrenal scanning agent, NP-59

    International Nuclear Information System (INIS)

    Sarkar, S.D.; Beierwaltes, W.H.; Ice, R.D.; Basmadjian, G.P.; Hertzel, K.R.; Kennedy, W.P.; Mason, M.M.

    1975-01-01

    The first synthesis of 131 I-19-iodocholesterol had a 10 to 25 percent radiochemical impurity that was not iodide ion. This impurity has been identified as 6β- 131 I-iodomethyl-19-nor cholest-5(10)-en-3β-ol (NP-59) and has been synthesized. Tissue distribution studies with 131 I-NP-59 in rats and dogs revealed a higher adrenal uptake and adrenal-to-tissue ratios compared to 131 I 19-iodocholesterol, probably less in vivo deiodination, and superior adrenal images. A high uptake was seen in the adrenal medulla in addition to that in the cortex. Iodine-131-NP-59 is being evaluated for the early detection of adrenal--cortical disorders and as a potential scanning agent for detecting structural abnormalities of the adrenal medulla

  6. Conjunctival lymphoma arising from reactive lymphoid hyperplasia

    Directory of Open Access Journals (Sweden)

    Fukuhara Junichi

    2012-09-01

    Full Text Available Abstract Extra nodal marginal zone B-cell lymphoma (EMZL of the conjunctiva typically arises in the marginal zone of mucosa-associated lymphoid tissue. The pathogenesis of conjunctival EMZL remains unknown. We describe an unusual case of EMZL arising from reactive lymphoid hyperplasia (RLH of the conjunctiva. A 35-year-old woman had fleshy salmon-pink conjunctival tumors in both eyes, oculus uterque (OU. Specimens from conjunctival tumors in the right eye, oculus dexter (OD, revealed a collection of small lymphoid cells in the stroma. Immunohistochemically, immunoglobulin (Ig light chain restriction was not detected. In contrast, diffuse atypical lymphoid cell infiltration was noted in the left eye, oculus sinister (OS, and positive for CD20, a marker for B cells OS. The tumors were histologically diagnosed as RLH OD, and EMZL OS. PCR analysis detected IgH gene rearrangement in the joining region (JH region OU. After 11 months, a re-biopsy specimen demonstrated EMZL based on compatible pathological and genetic findings OD, arising from RLH. This case suggests that even if the diagnosis of the conjunctival lymphoproliferative lesions is histologically benign, confirmation of the B-cell clonality by checking IgH gene rearrangement should be useful to predict the incidence of malignancy.

  7. Clinical and pathological aspects of epithelial hyperplasia.

    Science.gov (United States)

    Gîrtan, Mihaela; Stăniceanu, Florica; Zurac, Sabina; Laba, Elisabeta; Forna, Norina

    2008-01-01

    In many cases, the oral health status indicates the general status of the body. 90% of the disorders of the body also manifest at the level of the oral cavity, which means that the dentist can draw the attention of a certain health problem. Diabetes mellitus is associated with a high prevalence of the lesions of the oral mucous, especially lichen planus, recurrent aphthous stomatitis or oral candidiasis. We present here a case of diabetes mellitus with hyperplasic lesion at the level of the inferior vestibule, extended to the right jugal mucosa. The lesion appeared pursuant to the application of removable prosthetics. The biopsy specimen was examined using normal and special staining (HE Hematoxiline - eosine, Van Gieson VG) and immunohistochemistry (IHC). In the HE stain, an epithelial hyperplasia was noticed as a result of the proliferation of the basal cells, associated with hyperkeratosis (parakeratosis or orthokeratosis). A moderated inflammatory limphoplasmocitary infiltrate, composed by lymphocytes and plasma cells, was present within the hyperplasic chorion. The immunohistochemical reactions revealed Ki-67 positive nuclei in the basal and suprabasal strata (indicating an increased proliferating activity); rare p53 positive nuclei in the basal stratum (indicating a suppressive action on the cell proliferation); CD3/CD8 positive cells in the inflammatory infiltrate (indicating an important number of T suppressor lymphocytes in the inflammatory infiltrate). In conclusion, diabetes mellitus is a disease which frequently determines major modifications at the level of the oral cavity. Interdisciplinary collaboration between the pathologist and the dentist is necessary for adequate diagnosis and successful treatment.

  8. Transurethral microwave thermotherapy for benign prostatic hyperplasia

    Directory of Open Access Journals (Sweden)

    Rubeinstein Jonathan N.

    2003-01-01

    Full Text Available Transurethral resection of the prostate (TURP remains the gold standard for treatment of benign prostatic hyperplasia (BPH. In general, while this procedure is safe, patients require a spinal, epidural, or general anesthesia and often several days of hospital stay; the potential morbidity and mortality limits the use of TURP in high-risk patients. Pharmacotherapy has been recommended as a first-line therapy for all patients with mild to moderate symptoms. Patients are oftentimes enthusiastic if they are offered a one-time method to treat lower urinary tract symptoms secondary to BPH, provided that the method offers reduced risk and allows an efficacy equal to that of medical therapy. One such method is transurethral microwave thermotherapy (TUMT. TUMT involves the insertion of a specially designed urinary catheter with a microwave antenna, which heats the prostate and destroys hyperplastic prostate tissue. TUMT allows the avoidance of general or regional anesthesia, and results in minimal blood loss and fluid absorption. In this review, the authors discussed the current indications and outcome of TUMT, including the history of the procedure, the mechanism of action, the indications for TUMT, the pre-operative considerations, the patient selection, the results in terms of efficacy, by comparing TUMT vs. Sham, TUMT vs. Alpha-blocker and TUMT vs. TURP. Finally, the complications are presented, as well as other uses and future directions of the procedure. The authors concluded that TUMT is a safe and effective minimally invasive alternative to treatment of symptomatic BPH.

  9. Benign prostatic hyperplasia: epidemiology, economics and evaluation.

    Science.gov (United States)

    Vuichoud, Camille; Loughlin, Kevin R

    2015-10-01

    Benign prostatic hyperplasia (BPH) is arguably the most common benign disease of mankind. As men age, the prostate inexorably grows often causing troubling symptoms causing them to seek out care. While traditionally treated by transurethral resection or open surgical removal of the hypertrophied adenoma, today the urologist has numerous medical, surgical and minimally invasive techniques available. In this supplement The Canadian Journal of Urology provides a review of the various techniques and medications available today. As an introduction to the supplement, the aim of this article is to review the epidemiology and economy of BPH as well as its natural history and diagnosis. A systematic review of available literature was looking for articles on BPH and its epidemiology, economics, natural history and management using PubMed database. The prevalence of this condition is increasing with the population aging and so does the economic burden. The exact etiology of this condition is unknown, but some risk factors have been identified. The diagnostic and treatment of this very common disease should rely on a strong collaboration between primary care physician and urologist. There are multiple options in treating BPH including medical, surgical and newer minimally invasive options. The challenge with having a variety of options is to review them with the patient and help the patient select the best treatment option for their condition.

  10. Löwenheim-Skolem theorems for non-classical first-order algebraizable logics

    Czech Academy of Sciences Publication Activity Database

    Dellunde, P.; García-Cerdaña, A.; Noguera, Carles

    2016-01-01

    Roč. 24, č. 3 (2016), s. 321-345 ISSN 1367-0751 R&D Projects: GA ČR GA13-14654S Institutional support: RVO:67985556 Keywords : Löwenheim-Skolem theorems * first-order predicate logic s * non-classical logic s * algebraizable logic s * model theory Subject RIV: BA - General Mathematics Impact factor: 0.575, year: 2016 http://library.utia.cas.cz/separaty/2016/MTR/noguera-0469168.pdf

  11. Phase-space representation of non-classical behaviour of scalar wave-fields

    Energy Technology Data Exchange (ETDEWEB)

    Canas-Cardona, Gustavo; Castaneda, Roman [Physics School, Universidad Nacional de Colombia Sede Medellin, A.A. 3840 Medellin (Colombia); Vinck-Posada, Herbert, E-mail: gcanas@unal.edu.co [Physics Department, Universidad Nacional de Colombia Sede Bogota, Bogota D.C (Colombia)

    2011-01-01

    The modelling of optical fields by using radiant and virtual point sources for the spatial coherence wavelets in the phase-space representation evidences some effects, conventionally attributed to non-classical correlations of light, although such type of correlations are not explicitly included in the model. Specifically, a light state is produced that has similar morphology to the Wigner Distribution Function of the well-known quantum Schroedinger cat and squeezed states.

  12. Electron microscopy of crystalline solids and non-classical crystal growth

    OpenAIRE

    Greer, Heather Frances

    2013-01-01

    This project concerns the non-classical crystal growth of various porous and non-porous materials. In order to determine their crystal growth mechanism, the reaction was stopped at several different reaction times with the size, morphology, crystal structure and orientation of the particles analysed using scanning electron microscopy and high resolution transmission electron microscopy as the principal characterisation techniques. Other techniques used include X-ray diffraction, energy disper...

  13. Non-classical austenite-martensite interfaces observed in single crystals of Cu-Al-Ni

    Czech Academy of Sciences Publication Activity Database

    Seiner, Hanuš; Landa, Michal

    2009-01-01

    Roč. 82, č. 11 (2009), s. 793-807 ISSN 0141-1594 R&D Projects: GA AV ČR(CZ) IAA200100627; GA ČR(CZ) GP202/09/P164 Institutional research plan: CEZ:AV0Z20760514 Keywords : shape memory alloy s * martensitic microstructure * non-classical interfaces * crossing twins Subject RIV: BJ - Thermodynamics Impact factor: 0.935, year: 2009 http://www.informaworld.com

  14. Observation of non-classical rotational inertia in bulk solid 4He

    OpenAIRE

    Kondo, Motoshi; Takada, Shunichi; Shibayama, Yoshiyuki; Shirahama, Keiya

    2006-01-01

    In recent torsional oscillator experiments by Kim and Chan (KC), a decrease of rotational inertia has been observed in solid 4He in porous materials and in a bulk annular channel. This observation strongly suggests the existence of "non-classical rotational inertia" (NCRI), i.e. superflow, in solid 4He. In order to study such a possible "supersolid" phase, we perform torsional oscillator experiments for cylindrical solid 4He samples. We have observed decreases of rotational inertia below 200 ...

  15. Nonsuperfluid origin of the nonclassical rotational inertia in a bulk sample of solid 4He.

    Science.gov (United States)

    Reppy, John D

    2010-06-25

    The torsional oscillator experiments described here examine the effect of disorder on the nonclassical rotational inertia (NCRI) of a solid 4He sample. The NCRI increases with increasing disorder, but the period changes responsible for this increase occur primarily at higher temperatures. Contrary to expectations based on a supersolid scenario, the oscillator period remains relatively unaffected at the lowest temperatures. This result points to a nonsuperfluid origin for the NCRI.

  16. Cinacalcet HCl prevents development of parathyroid gland hyperplasia and reverses established parathyroid gland hyperplasia in a rodent model of CKD

    OpenAIRE

    Miller, Gerald; Davis, James; Shatzen, Edward; Colloton, Matthew; Martin, David; Henley, Charles M.

    2011-01-01

    Background. Secondary hyperparathyroidism (sHPT) represents an adaptive response to progressively impaired control of calcium, phosphorus and vitamin D in chronic kidney disease (CKD). It is characterized by parathyroid hyperplasia and excessive synthesis and secretion of parathyroid hormone (PTH). Parathyroid hyperplasia in uremic rats can be prevented by calcium-sensing receptor (CaSR) activation with the calcimimetic cinacalcet (Sensipar®/Mimpara®); however, it is unknown, how long the eff...

  17. Idiopathic Spontaneous Adrenal Hemorrhage in the Third Trimester of Pregnancy

    Directory of Open Access Journals (Sweden)

    Narin Nasiroglu Imga

    2013-01-01

    Full Text Available Spontaneous adrenal hemorrhage (SAH is seen in the absence of trauma or adrenal tumor in adrenal glands. The incidence of SAH has been reported from 0.14% to 1.1% and it usually involves the right gland. During pregnancy, idiopathic unilateral SAH has been reported rarely. We present a case which comes to emergency department with an acute abdominal pain and the test results showed spontaneous left SAH.

  18. [A non-classical approach to medical practices: Michel Foucault and Actor-Network Theory].

    Science.gov (United States)

    Bińczyk, E

    2001-01-01

    The text presents an analysis of medical practices stemming from two sources: Michel Foucault's conception and the research of Annemarie Mol and John Law, representatives of a trend known as Actor-Network Theory. Both approaches reveal significant theoretical kinship: they can be successfully consigned to the framework of non-classical sociology of science. I initially refer to the cited conceptions as a version of non-classical sociology of medicine. The identity of non-classical sociology of medicine hinges on the fact that it undermines the possibility of objective definitions of disease, health and body. These are rather approached as variable social and historical phenomena, co-constituted by medical practices. To both Foucault and Mol the main object of interest was not medicine as such, but rather the network of medical practices. Mol and Law sketch a new theoretical perspective for the analysis of medical practices. They attempt to go beyond the dichotomous scheme of thinking about the human body as an object of medical research and the subject of private experience. Research on patients suffering blood-sugar deficiency provide the empirical background for the thesis of Actor-Network Theory representatives. Michel Foucault's conceptions are extremely critical of medical practices. The French researcher describes the processes of 'medicalising' Western society as the emergence of a new type of power. He attempts to sensitise the reader to the ethical dimension of the processes of medicalising society.

  19. Conformational variation in structures of classical and nonclassical MHCII proteins and functional implications

    Science.gov (United States)

    Painter, Corrie A.; Stern, Lawrence J.

    2012-01-01

    Summary Recent structural characterizations of classical and nonclassical major histocompatibility complex class II (MHCII) proteins have provided a view into the dynamic nature of the MHCII peptide binding groove and the role that structural changes play in peptide loading processes. Although there have been numerous reports of crystal structures for MHCII-peptide complexes, a detailed analysis comparing all the structures has not been reported, and subtle conformational variations present in these structures may not have been fully appreciated. We compared the 91 MHCII crystal structures reported in the PDB to date, including a HLA-DR mutant particularly susceptible to DM-mediated peptide exchange, and reviewed experimental and computational studies of the effect of peptide binding on MHCII structure. These studies provide evidence for conformational lability in and around the α subunit 310 helix at residues α48-51, a region known to be critical for HLA-DM-mediated peptide exchange. A biophysical study of MHC-peptide hydrogen bond strengths and a recent structure of the nonclassical MHCII protein HLA-DO reveal changes in the same region. Conformational variability was observed also in the vicinity of a kink in the β subunit helical region near residue β66 and in the orientation and loop conformation in the β2 Ig domain. Here, we provide an overview of the regions within classical and nonclassical MHCII proteins that display conformational changes and the potential role that these changes may have in the peptide loading/exchange process. PMID:23046127

  20. The prognostic role of classical and nonclassical MHC class I expression in endometrial cancer.

    Science.gov (United States)

    Bijen, Claudia B M; Bantema-Joppe, Enja J; de Jong, Renske A; Leffers, Ninke; Mourits, Marian J E; Eggink, Henk F; van der Zee, Ate G J; Hollema, Harry; de Bock, Geertruida H; Nijman, Hans W

    2010-03-15

    The aim of this study was to investigate classical MHC class I and nonclassical MHC (human leukocyte antigen-G [HLA-G]) expression in a large cohort of patients with endometrial cancer, to determine the prognostic value of these cell surface markers and their relation with clinicopathological variables. Tissue microarrays containing epithelial endometrial carcinoma tissue from 554 patients were stained for classical and nonclassical MHC class I using the following monoclonal antibodies: 4H84 (anti-HLA-G), beta2-m (anti-beta-2-microglobulin) and HC-10 (MHC class I antigen heavy chain). Expression data were linked to known clinicopathological characteristics and survival. HLA-G upregulation and MHC class I downregulation in neoplastic cells was observed in 40% and 48%, respectively. Nonendometrioid tumor type, advanced stage disease (FIGO stage > or = II) and poorly or undifferentiated tumors were associated with MHC class I downregulation. Absence of HLA-G expression was independently associated with MHC class I downregulation. In univariate analysis, MHC class I downregulation was a predictor of worse disease-specific survival. Prognostic unfavorable tumor characteristics were correlated with downregulation of MHC class I expression in endometrial cancer cells. Furthermore, downregulated MHC class I has a negative impact on disease-specific survival, observed in a large cohort of patients with endometrial cancer. As there seems to be a relation between classical and nonclassical MHC class I molecules (HLA-G), further research is warranted to unravel this regulatory mechanism.

  1. Cinacalcet HCl prevents development of parathyroid gland hyperplasia and reverses established parathyroid gland hyperplasia in a rodent model of CKD

    Science.gov (United States)

    Miller, Gerald; Davis, James; Shatzen, Edward; Colloton, Matthew; Martin, David

    2012-01-01

    Background. Secondary hyperparathyroidism (sHPT) represents an adaptive response to progressively impaired control of calcium, phosphorus and vitamin D in chronic kidney disease (CKD). It is characterized by parathyroid hyperplasia and excessive synthesis and secretion of parathyroid hormone (PTH). Parathyroid hyperplasia in uremic rats can be prevented by calcium-sensing receptor (CaSR) activation with the calcimimetic cinacalcet (Sensipar®/Mimpara®); however, it is unknown, how long the effects of cinacalcet persist after withdrawal of treatment or if cinacalcet is efficacious in uremic rats with established sHPT. Methods. We sought to determine the effect of cinacalcet discontinuation in uremic rats and whether cinacalcet was capable of influencing parathyroid hyperplasia in animals with established sHPT. Results. Discontinuation of cinacalcet resulted in reversal of the beneficial effects on serum PTH and parathyroid hyperplasia. In rats with established sHPT, cinacalcet decreased serum PTH and mediated regression of parathyroid hyperplasia. The cinacalcet-mediated decrease in parathyroid gland size was accompanied by increased expression of the cyclin-dependent kinase inhibitor p21. Prevention of cellular proliferation with cinacalcet occurred despite increased serum phosphorus and decreased serum calcium. Conclusions. The animal data provided suggest established parathyroid hyperplasia can be reversed by modulating CaSR activity with cinacalcet and that continued treatment may be necessary to maintain reductions in PTH. PMID:22036941

  2. Cinacalcet HCl prevents development of parathyroid gland hyperplasia and reverses established parathyroid gland hyperplasia in a rodent model of CKD.

    Science.gov (United States)

    Miller, Gerald; Davis, James; Shatzen, Edward; Colloton, Matthew; Martin, David; Henley, Charles M

    2012-06-01

    Secondary hyperparathyroidism (sHPT) represents an adaptive response to progressively impaired control of calcium, phosphorus and vitamin D in chronic kidney disease (CKD). It is characterized by parathyroid hyperplasia and excessive synthesis and secretion of parathyroid hormone (PTH). Parathyroid hyperplasia in uremic rats can be prevented by calcium-sensing receptor (CaSR) activation with the calcimimetic cinacalcet (Sensipar®/Mimpara®); however, it is unknown, how long the effects of cinacalcet persist after withdrawal of treatment or if cinacalcet is efficacious in uremic rats with established sHPT. We sought to determine the effect of cinacalcet discontinuation in uremic rats and whether cinacalcet was capable of influencing parathyroid hyperplasia in animals with established sHPT. Discontinuation of cinacalcet resulted in reversal of the beneficial effects on serum PTH and parathyroid hyperplasia. In rats with established sHPT, cinacalcet decreased serum PTH and mediated regression of parathyroid hyperplasia. The cinacalcet-mediated decrease in parathyroid gland size was accompanied by increased expression of the cyclin-dependent kinase inhibitor p21. Prevention of cellular proliferation with cinacalcet occurred despite increased serum phosphorus and decreased serum calcium. The animal data provided suggest established parathyroid hyperplasia can be reversed by modulating CaSR activity with cinacalcet and that continued treatment may be necessary to maintain reductions in PTH.

  3. Cytochrome P450-catalyzed binding of 3-methylsulfonyl-DDE and o,p'-DDD in human adrenal zona fasciculata/reticularis.

    Science.gov (United States)

    Lindhe, Orjan; Skogseid, Britt; Brandt, Ingvar

    2002-03-01

    3-Methylsulfonyl-2,2'-bis(4-chlorophenyl)-1,1'-dichloroethene (MeSO(2)-DDE) is a potent, tissue-specific toxicant that induces necrosis of the adrenal zona fasciculata following a local CYP11B1-catalyzed activation to a reactive intermediate in mice. Autoradiography was used to examine CYP11B1-catalyzed binding of MeSO(2)-[(14)C]DDE and the adrenocorticolytic drug 2-(2-chlorophenyl)-2-(4-chlorophenyl)-1,1-dichlorethane; (o,p'-[(14)C]DDD, Mitotane, Lysodren) in human adrenal tissue slice culture. Both compounds gave rise to a selective binding in the one sample of normal adrenal zona fasciculata/reticularis, leaving zona glomerulosa and the adrenal medulla devoid of binding. Addition of the CYP11B1 selective inhibitor metyrapone (50 microM) reduced MeSO(2)-[(14)C]DDE binding below the detection limit, whereas o,p'-[(14)C]DDD binding was reduced only by 42%. Selective binding of MeSO(2)-[(14)C]DDE and o,p'-[(14)C]DDD was also observed in an aldosterone-producing adrenocortical carcinoma and in a nonfunctional adrenocortical hyperplasia. Exposure of slices from the normal adrenal cortex to MeSO(2)-DDE (25 microM) resulted in an increased accumulation of 11-deoxycorticosterone, 11-deoxycortisol and androstenedione in the medium, and exposure to o,p'-DDD (25 microM) did not alter the steroid secretion pattern. No histological changes were found in either MeSO(2)-DDE- or o,p'-DDD-exposed slices, compared with nonexposed slices. We suggest that MeSO(2)-DDE might act as a potent adrenocorticolytic agent in humans. Further studies are needed to establish the usefulness of MeSO(2)-DDE as a possible alternative for the treatment of adrenocortical hypersecretion and tumor growth.

  4. Clinical evaluation of adrenal computed tomography and scintigraphy

    International Nuclear Information System (INIS)

    Hayasaka, Kazumasa; Yoshikawa, Hiroyuki; Asano, Akira; Kikuchi, Yuzo; Amo, Kazuo

    1983-01-01

    In 15 cases with adrenal lesion, we studied the clinical usefulness of computed tomography (CT) and scintigram. CT and RI have been successfully to locate adrenal funtioning cortical tumors (6/6) as small as 10 mm in diameter. In 5 adrenal non-funtioning cortical and medullary disorders, RI finding only shows RI activity is decreasing, but CT may be helpful in differential diagnosis. At present, CT is a reliable technique for locating adrenal disorders, and we should suggest that it should be the initial radiographic investigation. (author)

  5. Adrenal scintiscanning with NP-59: a new radioiodinated cholesterol agent

    International Nuclear Information System (INIS)

    Miles, J.M.; Wahner, H.W.; Carpenter, P.C.; Salassa, R.M.; Northcutt, R.C.

    1979-01-01

    Adrenal imaging in the past has been limited in its clinical application by the long interval between administration of dose and visualization of adrenal glands. We review our experience with the use of a newer labeling agent, NP-59, in 29 patients with various adrenal disorders and in 4 normal subjects. With this agent, identification of adrenal lesions is possible with a high degree of accuracy, and diagnostic information is usually available within 48 hrs. NP-59 is particularly useful in evaluating primary aldosteronism and selected cases of Cushing's syndrome

  6. Detailed examination of the adrenal glands by angiography and radioimmunologic measurement of hormones in adrenal venous blood

    International Nuclear Information System (INIS)

    Yugrinov, O.G.; Slavnov, V.N.; Komissarenko, I.V.; Olejnik, V.A.; Benikova, E.A.

    1984-01-01

    In 222 patients the adrenal glands were examined in detail by arteriography and venography, and if indicated also the ovaries, kidneys, bladder and other organs were checked up. Blood samples were taken from the adrenal glands, renal veins and the vena cava inferior in the bifurcational and subdiaphragmatic region. According to the clinical requirements cortisol, corticotropine, aldosterone, adrenaline, noradrenaline and renine activity were determined. Comprehensive angiographic and radioimmunologic studies revealed in 54 patients tumors of the adrenal cortex. Tumors of the adrenal medulla were detected in 43 of the cases. In 103 cases a morbus Icenko-Cushing was found. The basic examination of the diagnostic schedule was selective adrenal venography. Adrenal arteriography and measurement of venous hormone levels were complementary investigations and were rarely used as independent methods. (author)

  7. Contemporary surgical treatment of benign prostatic hyperplasia.

    Science.gov (United States)

    Nunes, Ricardo Luís Vita; Antunes, Alberto Azoubel; Constantin, Davi Souza

    2017-08-01

    Benign prostatic hyperplasia (BPH) is a common condition in adult men and its incidence increases progressively with aging. It has an important impact on the individual's physical and mental health and its natural progression can lead to serious pathological situations. Although the initial treatment is pharmacological, except in specific situations, the tendency of disease progression causes a considerable portion of the patients to require surgical treatment. In this case, there are several options available today in the therapeutic armamentarium. Among the options, established techniques, such as open surgery and endoscopic resection using monopolar energy, still prevail in the choice of surgeons because they are more accessible, both from a socioeconomic standpoint in the vast majority of medical services and in terms of training of medical teams. On the other hand, new techniques and technologies arise sequentially in order to minimize aggression, surgical time, recovery and complications, optimizing results related to the efficacy/safety dyad. Each of these techniques has its own peculiarities regarding availability due to cost, learning curve and scientific consolidation in order to achieve recognition as a cutting-edge method in the medical field. The use of bipolar energy in endoscopic resection of the prostate, laser vaporization and enucleation techniques, and videolaparoscopy are examples of new options that have successfully traced this path. Robot-assisted surgery has gained a lot of space in the last decade, but it still needs to dodge the trade barrier. Other techniques and technologies will need to pass the test of time to be able to conquer their space in this growing market.

  8. Chromosomal aberrations in benign prostatic hyperplasia patients

    Directory of Open Access Journals (Sweden)

    Muammer Altok

    2016-01-01

    Full Text Available Purpose: To investigate the chromosomal changes in patients with benign prostatic hyperplasia (BPH. Materials and Methods: A total of 54 patients diagnosed with clinical BPH underwent transurethral prostate resection to address their primary urological problem. All patients were evaluated by use of a comprehensive medical history and rectal digital examination. The preoperative evaluation also included serum prostate-specific antigen (PSA measurement and ultrasonographic measurement of prostate volume. Prostate cancer was detected in one patient, who was then excluded from the study. We performed conventional cytogenetic analyses of short-term cultures of 53 peripheral blood samples obtained from the BPH patients. Results: The mean (±standard deviation age of the 53 patients was 67.8±9.4 years. The mean PSA value of the patients was 5.8±7.0 ng/mL. The mean prostate volume was 53.6±22.9 mL. Chromosomal abnormalities were noted in 5 of the 53 cases (9.4%. Loss of the Y chromosome was the most frequent chromosomal abnormality and was observed in three patients (5.7%. There was no statistically significant relationship among age, PSA, prostate volume, and chromosomal changes. Conclusions: Loss of the Y chromosome was the main chromosomal abnormality found in our study. However, this coexistence did not reach a significant level. Our study concluded that loss of the Y chromosome cannot be considered relevant for the diagnosis of BPH as it is for prostate cancer. Because BPH usually occurs in aging men, loss of the Y chromosome in BPH patients may instead be related to the aging process.

  9. Oxidative stress promotes benign prostatic hyperplasia.

    Science.gov (United States)

    Vital, Paz; Castro, Patricia; Ittmann, Michael

    2016-01-01

    Benign prostatic hyperplasia (BPH) is characterized by increased tissue mass in the transition zone of the prostate, which leads to obstruction of urine outflow and significant morbidity in the majority of older men. Plasma markers of oxidative stress are increased in men with BPH but it is unclear whether oxidative stress and/or oxidative DNA damage are causal in the pathogenesis of BPH. Levels of 8-OH deoxyguanosine (8-OH dG), a marker of oxidative stress, were measured in prostate tissues from normal transition zone and BPH by ELISA. 8-OH dG was also detected in tissues by immunohistochemistry and staining quantitated by image analysis. Nox4 promotes the formation of reactive oxygen species. We therefore created and characterized transgenic mice with prostate specific expression of Nox4 under the control of the prostate specific ARR2PB promoter. Human BPH tissues contained significantly higher levels of 8-OH dG than control transition zone tissues and the levels of 8-OH dG were correlated with prostate weight. Cells with 8-OH dG staining were predominantly in the epithelium and were present in a patchy distribution. The total fraction of epithelial staining with 8-OH dG was significantly increased in BPH tissues by image analysis. The ARR2PB-Nox4 mice had increased oxidative DNA damage in the prostate, increased prostate weight, increased epithelial proliferation, and histological changes including epithelial proliferation, stromal thickening, and fibrosis when compared to wild type controls. Oxidative stress and oxidative DNA damage are important in the pathogenesis of BPH. © 2015 Wiley Periodicals, Inc.

  10. Computed tomography findings in diseases of the adrenal gland

    International Nuclear Information System (INIS)

    Ozturk, E.; Sildiroglu, H.O.; Sonmez, G.; Basekim, C.C.; Kantarci, M.; Gueven, F.; Doganay, S.; Bozkurt, M.

    2009-01-01

    The adrenal gland is a common site of disease, with an abnormality prevalence as high as 9% in autopsy series. With the increasing use of CT, adrenal lesions are frequently found in the daily practice of radiology and are diagnosed in up to 5% of CT examinations performed for varied reasons. Imaging features on CT can establish a specific diagnosis of many of these lesions, including myelolipoma, hematoma and cysts. Once a diagnostic dilemma, now adenomas can be accurately diagnosed using unenhanced CT, chemical shift magnetic resonance imaging and CT contrast washout analysis. Because the adrenal gland is also a frequent site of metastasis, recent literature has focused on imaging characterization of adrenal masses for differentiation of adenomas from metastases. In patients without known malignancy, most adrenal lesions are benign and a specific diagnosis can now be made on the basis of imaging features. It is important to understand the prevalence of adrenal abnormalities because the gland is a common site of disorders, and the increased use of cross-sectional imaging has increased the frequency of detection of adrenal lesions. The prevalence of disease is important in predicting the risk of malignancy when an adrenal mass is discovered in a patient without known cancer. Detection of adrenal gland diseases has increased substantially with the advent and widespread use of imaging techniques. Although several imaging modalities can be used, CT has a central role in both detection and differential diagnosis of an adrenal lesion. The aim of this article is to review the CT findings of adrenal gland diseases. (author) [de

  11. Ramadan fasting in patients with adrenal insufficiency.

    Science.gov (United States)

    Chihaoui, Mélika; Chaker, Fatma; Yazidi, Meriem; Grira, Wafa; Ben Amor, Zohra; Rejeb, Ons; Slimane, Hedia

    2017-01-01

    The risks of fasting during Ramadan in patients with adrenal insufficiency are unknown. The aims of this study were to evaluate these risks in such patients, to determine the risk factors and finally to set some recommendations. It is a cross-sectional study about 180 patients with known and treated adrenal insufficiency. The patients responded to a 14-item questionnaire concerning their knowledge about the disease and fasting during the last month of Ramadan. There were 132 women and 48 men. The mean age was 47.6 ± 15.0 years (14-79). One hundred and thirty eight patients (76.7 %) were advised by their physician not to fast. Ninety-one patients (50.5 %) tried to fast. Complications occurred in 61 cases (67.0 %): asthenia in 88.5 % of cases, intense thirst in 32.8 %, symptoms of dehydration in 49.2 % and symptoms of hypoglycaemia in 18 %. One patient was hospitalized. Fifty-five patients (60.4 %) were able to fast for the whole month. Age, gender, duration of the disease, its primary origin, associated hypothyroidism, diabetes mellitus, hypertension or diabetes insipidus and daily dose of hydrocortisone did not significantly differ between fasters and non-fasters, full-month-fasters and partial-month-fasters, and fasters with complications and fasters without complications. The frequency of adequate knowledge about the disease was significantly higher in full-month-fasters vs. partial-month-fasters, and in fasters without complications vs. those with complications. In patients with adrenal insufficiency, fasting can cause complications especially if the level of knowledge about the disease is low.

  12. Renal Cell Carcinoma Mimicking Adrenal Tumor

    Directory of Open Access Journals (Sweden)

    Mohammad Kazem Moslemi

    2010-01-01

    Full Text Available There are a variety of causes of adrenal pseudotumors on computerized tomography (CT scan, including upper-pole renal mass, gastric diverticulum, prominent splenic lobulation, pancreatic mass, hepatic mass, and periadrenal varices. We present a case of a large subhepatic mass that discrimination of its origin from neighborhood organs was difficult preoperatively. Our patient was a 58 years old man, that three months after an unsuccessful operation in another center for a pseudoadrenal mass underwent a very difficult subcapsular tumorectomy in our center.

  13. Septicaemia and adrenal haemorrhage in congenital asplenia.

    Science.gov (United States)

    Dyke, M P; Martin, R P; Berry, P J

    1991-01-01

    Five patients developed overwhelming infection as a result of congenital asplenia, which was previously unsuspected in all cases. Each illness followed a fulminant course resulting in death within 24 hours. They illustrate the respective roles of Haemophilus influenzae infection (n = 4) and adrenal haemorrhage (n = 4) in this condition. We suggest a management protocol for screening infants with abnormalities of the atria or viscera with splenic ultrasound and examination of a blood film for Howell-Jolly bodies. Vaccination and prophylactic antibiotics should be considered for those at risk. Vigorous use of parenteral antibiotics and steroids in suspected infection is recommended. PMID:2039257

  14. Incidence and Cause of Hypertension During Adrenal Radiofrequency Ablation

    Energy Technology Data Exchange (ETDEWEB)

    Yamakado, Koichiro, E-mail: yama@clin.medic.mie-u.ac.jp; Takaki, Haruyuki [Mie University School of Medicine, Department of Interventional Radiology (Japan); Yamada, Tomomi [Mie University School of Medicine, Department of Translational Medicine (Japan); Yamanaka, Takashi; Uraki, Junji; Kashima, Masataka; Nakatsuka, Atsuhiro; Takeda, Kan [Mie University School of Medicine, Department of Interventional Radiology (Japan)

    2012-12-15

    Purpose: To evaluate the incidence and cause of hypertension prospectively during adrenal radiofrequency ablation (RFA). Methods: For this study, approved by our institutional review board, written informed consent was obtained from all patients. Patients who received RFA for adrenal tumors (adrenal ablation) and other abdominal tumors (nonadrenal ablation) were included in this prospective study. Blood pressure was monitored during RFA. Serum adrenal hormone levels including epinephrine, norepinephrine, dopamine, and cortisol levels were measured before and during RFA. The respective incidences of procedural hypertension (systolic blood pressure >200 mmHg) of the two patient groups were compared. Factors correlating with procedural systolic blood pressure were evaluated by regression analysis.ResultsNine patients underwent adrenal RFA and another 9 patients liver (n = 5) and renal (n = 4) RFA. Asymptomatic procedural hypertension that returned to the baseline by injecting calcium blocker was found in 7 (38.9%) of 18 patients. The incidence of procedural hypertension was significantly higher in the adrenal ablation group (66.7%, 6/9) than in the nonadrenal ablation group (11.1%, 1/9, P < 0.0498). Procedural systolic blood pressure was significantly correlated with serum epinephrine (R{sup 2} = 0.68, P < 0.0001) and norepinephrine (R{sup 2} = 0.72, P < 0.0001) levels during RFA. The other adrenal hormones did not show correlation with procedural systolic blood pressure. Conclusion: Hypertension occurs frequently during adrenal RFA because of the release of catecholamine.

  15. The Influence of Adrenal Secretons on Leucocyte Distribution and ...

    African Journals Online (AJOL)

    Many apparently, health Africans present with leuko-neutropenia. The origin of the so-called benign ethnic leuko-neutropenia remains uncertain. Africans are also, known to have small adrenal cortices. Cortisol is the major secretion of the adrenal cortex, which profoundly influences leukocyte profile. We investigated the ...

  16. Glucose metabolism and adrenal function in goats bred for fibre ...

    African Journals Online (AJOL)

    It has been proposed that the abortions, cold-stress fatalities, and slow growth rates typical of the South African type of Angora goat can be explained by congenital adrenal hypofunction incident to genetic selection for hair production. The aim of this experiment was to compare glucose metabolism and adrenal function in ...

  17. Adrenal insufficiency in critically ill septic patients at Dr George ...

    African Journals Online (AJOL)

    Rationale: Adrenal insufficiency occurs with varying frequency in critically ill patients. It is usually associated with a high mortality and poor clinical outcome. Objective: To determine the incidence of adrenal failure in patients with severe sepsis and septic shock admitted to our intensive care unit. Design: Prospective ...

  18. Extra-adrenal Pheochromocytoma: Experience in Mulago Hospital ...

    African Journals Online (AJOL)

    ... have a family history of malignancy. Management of extra-adrenal pheochromocytoma is enblock en-mass surgical resection. Chemotherapy, and radiation therapy may be necessary in malignant disease. Longterm follow-up is essential, as extra-adrenal pheochromocytomas can recur many years after initial diagnosis.

  19. An acute adrenal insufficiency revealing pituitary metastases of lung ...

    African Journals Online (AJOL)

    An acute adrenal insufficiency revealing pituitary metastases of lung cancer in an elderly patient. ... The hypothalamic-pituitary MRI showed a pituitary hypertrophy, a nodular thickening of the pituitary stalk. The chest X Rays ... Hence we concluded to a lung cancer with multiple pituitary and adrenal gland metastases.

  20. Histologic and Immunohistochemical classification of 41 bovine adrenal gland neoplasms

    DEFF Research Database (Denmark)

    Grossi, Anette Blak; Leifsson, Páll S.; Jensen, Henrik Elvang

    2013-01-01

    Tumors of the adrenal glands are among the most frequent tumors in cattle; however, few studies have been conducted to describe their characteristics. The aim of this study was to classify 41 bovine adrenal neoplasms from 40 animals based on macroscopic and histologic examination, including...

  1. Adrenal disorders: Is there Any role for vitamin D?

    Science.gov (United States)

    Tirabassi, Giacomo; Salvio, Gianmaria; Altieri, Barbara; Ronchi, Cristina L; Della Casa, Silvia; Pontecorvi, Alfredo; Balercia, Giancarlo

    2017-09-01

    An emerging branch of research is examining the linkage between Vitamin D and nonskeletal disorders, including endocrine diseases. In this regard, a still little studied aspect concerns the involvement of vitamin D in adrenal gland disorders. Adrenal gland disorders, which might be theoretically affected by vitamin D unbalance, include adrenal insufficiency, Cushing's syndrome, adrenocortical tumors and hyperaldosteronism. In this review, we provide an updated document, which tries to collect and discuss the limited evidence to be found in the literature about the relationship between vitamin D and adrenal disorders. We conclude that there is insufficient evidence proving a causal relationship between vitamin D levels and adrenal disorders. Evidence coming from cross-sectional clinical studies can hardly clarify what comes first between vitamin D unbalance and adrenal disease. On the other hand, longitudinal studies monitoring the levels of vitamin D in patients with adrenal disorders or, conversely, the possible development of adrenal pathologies in subjects affected by impaired vitamin D levels would be able to elucidate this still unclear issue.

  2. Clinical experience with 75Se selenomethylcholesterol adrenal imaging

    International Nuclear Information System (INIS)

    Shapiro, B.; Britton, K.E.; Hawkins, L.A.; Edwards, C.R.W.

    1981-01-01

    The results of quantitative adrenal imaging using 75 Se selenomethylcholesterol in sixty-two subjects are analysed. The adrenal area was localized by a renal scan, lateral views of which enabled adrenal depth to be estimated. The first nineteen cases were scanned with a rectilinear scanner and the remaining forty-three cases imaged with a gamma camera. Quantitation of adrenal uptake was performed on computer-stored static images obtained 7 and 14 days post-injection of 75 Se selenomethylcholesterol (3 and 6 days in the first ten cases studied). Normal uptake was found to be 0.07-0.30% of the administered dose. Overall predictive accuracy of the type of adrenal disorder of thirty-two patients with Cushing's syndrome was 90.6%. Overall predictive accuracy of the cause of Conn's syndrome in twenty-two cases was 86.4%. The mean uptake in the normal adrenal in cases of unilateral adenoma was 0.19% (range 0.07-0.30%). Causes of unsatisfactory adrenal imaging are examined. The procedure is recommended as the localizing and lateralizing technique of choice in Cushing's syndrome except where due to adrenal carcinoma, and as an important non-invasive technique in Conn's syndrome for the lateralization of adenoma. (author)

  3. Release of galanin from isolated perfused porcine adrenal glands

    DEFF Research Database (Denmark)

    Holst, J J; Ehrhart-Bornstein, M; Messell, T

    1991-01-01

    in anesthetized pigs increased the concentration of galanin in the caval vein but not in arterial plasma. It is concluded that galanin, coreleased with catecholamines from the adrenal glands, may have endocrine functions but that galanin may also have local regulatory functions in the adrenals....

  4. Volume of the adrenal and pituitary glands in depression

    DEFF Research Database (Denmark)

    Kessing, Lars Vedel; Willer, Inge Stoel; Knorr, Ulla

    2011-01-01

    Numerous studies have shown that the hypothalamic-pituitary-adrenal (HPA) axis is hyperactive in some depressed patients. It is unclear whether such hyperactivity results in changed volumes of the adrenal glands, pituitary gland and hypothalamus. We systematically reviewed all controlled studies...... on the adrenal or pituitary glands or hypothalamus volume in unipolar depressive disorder published in PubMed 1966 to December 2009. We identified three studies that investigated the volume of the adrenal glands and eight studies that examined the volume of the pituitary gland, but no studies on hypothalamus...... were found. Two out of three studies found a statistically significant increase in adrenal volume in patients compared to controls. Four out of eight studies found a statistically significant increase in pituitary volume in patients compared to controls. Different methodological problems were...

  5. Uncommon adrenal masses: CT and MRI features with histopathologic correlation

    International Nuclear Information System (INIS)

    Guo Yingkun; Yang Zhigang; Li Yuan; Deng Yuping; Ma Ensen; Min Pengqiu; Zhang Xiaochun

    2007-01-01

    Adrenal glands are common sites of diseases. With dramatically increased use of computed tomography (CT) and magnetic resonance (MR) imaging, more and more uncommon adrenal masses have been detected incidentally at abdominal examinations performed for other purposes. In this article, uncommon adrenal masses are classified as cystic masses (endothelial cysts, epithelial cysts, parasitic cysts, and pseudocysts), solid masses (ganglioneuroma, ganglioneuroblastoma, extramedullary plasmacytoma (EMP), neurilemmoma, and lymphoma), fat-containing masses (myelolipoma, teratoma), and infectious masses (tuberculoma), and the imaging features of these uncommon masses are demonstrated. Although most of these lesions do not have specific imaging features, some fat-containing masses and cystic lesions present with characteristic appearances, such as myelolipoma, teratoma, and hydatid. Combination with histopathologic characteristic of these uncommon masses of adrenal gland, radiological features of these lesions on CT and MR imaging can be accurately understood with more confidences. Moreover, CT and MRI are highly accurate in localization of uncommon adrenal masses, and useful to guide surgical treatments

  6. The place of imaging in exploration of the adrenal glands

    International Nuclear Information System (INIS)

    Cyteval, C.; Pradel, J.; Pujol, J.; Lamarque, J.L.; Jaffiol, C.; Krempf, M.; Charbonnel, B.; Peltier, P.; Chatal, J.F.

    1990-01-01

    Currently, the major method of adrenal gland imaging is computed tomography. This method allows demonstration of normal adrenals and the diagnosis of adrenal masses (if these are greater than 1 cm in diameter). The examination should be directed by clinical signs and known laboratory investigations. Computed tomography is therefore the first line investigation to perform. Certain lesions may be better demonstrated by other methods: MRI and MIBG scintigraphy offer a greater specificity in the investigation of pheochromocytomas. In addition, scintigraphy can identify possible ectopic tumours or recurrences. - Selective catheterisation of the adrenal veins allows aldosterone and cortisone secretions to be assayed. There remains the problem of the incidental finding of adrenal masses in either an asymptomatic patient or in the context of investigation of spread of a known cancer. These lesions may benefit from diagnostic percutaneous guided biopsy [fr

  7. Volume of the adrenal and pituitary glands in depression

    DEFF Research Database (Denmark)

    Kessing, Lars Vedel; Willer, Inge Stoel; Knorr, Ulla

    2011-01-01

    on the adrenal or pituitary glands or hypothalamus volume in unipolar depressive disorder published in PubMed 1966 to December 2009. We identified three studies that investigated the volume of the adrenal glands and eight studies that examined the volume of the pituitary gland, but no studies on hypothalamus......Numerous studies have shown that the hypothalamic-pituitary-adrenal (HPA) axis is hyperactive in some depressed patients. It is unclear whether such hyperactivity results in changed volumes of the adrenal glands, pituitary gland and hypothalamus. We systematically reviewed all controlled studies...... in enlarged adrenal and pituitary glands and it is suggested that prospective studies should be conducted with scanning during successive depressive episodes and periods of remission....

  8. Giant adrenal myelolipoma: Incidentaloma with a rare incidental association

    Directory of Open Access Journals (Sweden)

    Wani Nisar

    2010-01-01

    Full Text Available Adrenal myelolipoma is an unusual, benign and biochemically inactive tumor that is composed of mature adipose and hematopoietic tissue. It is usually diagnosed accidentally and nowadays much more frequently because of widespread use of ultrasonography, computed tomography (CT and magnetic resonance imaging. Adrenal myelolipoma is usually unilateral and asymptomatic, though known to be associated with obesity, hypertension, endocrinological disorders and some malignancies. We report herein two cases of right-sided giant adrenal myelolipoma diagnosed by multidetector-row CT. One patient was symptomatic because of a large mass in the right upper abdomen, which on imaging with CT was seen to be right adrenal myelolipoma. Another patient had a large left side Bochdalek hernia and right adrenal myelolipoma was incidentally discovered on CT.

  9. Congenital Hemifacial Hyperplasia: Clinical Presentation and Literature Review

    Directory of Open Access Journals (Sweden)

    Karpagavalli Shanmugasundaram

    2016-01-01

    Full Text Available Hemifacial hyperplasia is a rare congenital malformation characterized by noticeable unilateral excess development of hard and soft tissues of the face. Asymmetry in Congenital Hemifacial Hyperplasia (CHH is usually evident at birth and accentuated at the age of puberty. The affected side grows exponentially as compared to the unaffected side. Multiple tissue involvement has resulted due to etiological heterogeneity like heredity, chromosomal abnormalities, altered intrauterine environment, and endocrine dysfunctions. As this lesion is rarely seen in our routine clinical practice, we present a case of hemifacial hyperplasia with reported orofacial features that supplement existing clinical knowledge. This paper also adds knowledge to the readers regarding detailed investigation procedures which has complemented our diagnosis. Further emphasis has been placed on periodic approach to its diagnosis and multidisciplinary management following correct diagnosis.

  10. Mutations of the KRAS oncogene in endometrial hyperplasia and carcinoma.

    Directory of Open Access Journals (Sweden)

    Wiesława Niklińska

    2009-05-01

    Full Text Available The aim of this study was to examine the prevalence and clinicopathological significance of KRAS point mutation in endometrial hyperplasia and carcinoma. We analysed KRAS in 11 cases of complex atypical hyperplasia and in 49 endometrial carcinomas using polymerase chain reaction associated with restriction fragment length polymorphism (PCR-RFPL. Point mutations at codon 12 of KRAS oncogene were identified in 7 of 49 (14,3% tumor specimens and in 2 of 11 (18,2% hyperplasias. No correlation was found between KRAS gene mutation and age at onset, histology, grade of differentiation and clinical stage. We conclude that KRAS mutation is a relatively common event in endometrial carcinogenesis, but with no prognostic value.

  11. Neural control of adrenal medullary and cortical blood flow during hemorrhage

    International Nuclear Information System (INIS)

    Breslow, M.J.; Jordan, D.A.; Thellman, S.T.; Traystman, R.J.

    1987-01-01

    Hemorrhagic hypotension produces an increase in adrenal medullary blood flow and a decrease in adrenal cortical blood flow. To determine whether changes in adrenal blood flow during hemorrhage are neurally mediated, the authors compared blood flow responses following adrenal denervation (splanchnic nerve section) with changes in the contralateral, neurally intact adrenal. Carbonized microspheres labeled with 153 Gd, 114 In, 113 Sn, 103 Ru, 95 Nb or 46 Se were used. Blood pressure was reduced and maintained at 60 mmHg for 25 min by hemorrhage into a pressurized bottle system. Adrenal cortical blood flow decreased to 50% of control with hemorrhage in both the intact and denervated adrenal. Adrenal medullary blood flow increased to four times control levels at 15 and 25 min posthemorrhage in the intact adrenal, but was reduced to 50% of control at 3, 5, and 10 min posthemorrhage in the denervated adrenal. In a separate group of dogs, the greater splanchnic nerve on one side was electrically stimulated at 2, 5, or 15 Hz for 40 min. Adrenal medullary blood flow increased 5- to 10-fold in the stimulated adrenal but was unchanged in the contralateral, nonstimulated adrenal. Adrenal cortical blood flow was not affected by nerve stimulation. They conclude that activity of the splanchnic nerve profoundly affects adrenal medullary vessels but not adrenal cortical vessels and mediates the observed increase in adrenal medullary blood flow during hemorrhagic hypotension

  12. DOES HYPOGONADISM ON RESULTS TRANSURETHRAL RESECTION OF BENIGN PROSTATIC HYPERPLASIA?

    Directory of Open Access Journals (Sweden)

    A. V. Sigaev

    2013-01-01

    Full Text Available Influence of hypogonadism on the results of transurethral resection of the prostate (TURP in patients with benign prostatic hyperplasia (BPH remains unexplored. At the survey included 98 patients with benign prostatic hyperplasia who underwent TURP. Revealed that the postoperative period in patients characterized by a significant decrease in the level of performance testosteronemii in all cases, and against the background of hypogonadism accompanied by the development of more complications. Preoperative correction of hypogonadism for 2 weeks prior to surgery allows a 2-3 times lower risk of postoperative complications. 

  13. Thymic Hyperplasia after Lung Transplantation Imitating Posttransplant Lymphoproliferative Disorder

    Science.gov (United States)

    Steger, Christina Maria; Semsroth, Severin; Hager, Thomas; Rieker, Ralf; Müller, Ludwig

    2011-01-01

    Thymic hyperplasia is usually associated with the treatment of malignant tumours and is sometimes linked with endocrine diseases. For the first time, we report a case of thymic hyperplasia in a patient 2 years after bilateral lung transplantation. Contrast-enhanced chest CT scan was highly suspicious for a posttransplant lymphoma or thymoma. Therefore, the patient received total thymectomy. Excised specimens were sent to the Department of Pathology. Unexpectedly, the histological examination revealed hyperplastic thymic tissue without evidence for a posttransplant lymphoproliferative disorder or malignancy. PMID:23213605

  14. Thymic Hyperplasia after Lung Transplantation Imitating Posttransplant Lymphoproliferative Disorder

    Directory of Open Access Journals (Sweden)

    Christina Maria Steger

    2011-01-01

    Full Text Available Thymic hyperplasia is usually associated with the treatment of malignant tumours and is sometimes linked with endocrine diseases. For the first time, we report a case of thymic hyperplasia in a patient 2 years after bilateral lung transplantation. Contrast-enhanced chest CT scan was highly suspicious for a posttransplant lymphoma or thymoma. Therefore, the patient received total thymectomy. Excised specimens were sent to the Department of Pathology. Unexpectedly, the histological examination revealed hyperplastic thymic tissue without evidence for a posttransplant lymphoproliferative disorder or malignancy.

  15. Management of primary aldosteronism in patients with adrenal hemorrhage following adrenal vein sampling: A brief review with illustrative cases.

    Science.gov (United States)

    Hannah-Shmouni, Fady; Demidowich, Andrew; Alves, Beatriz Rizkallah; Paluch, Gabriela Dockhorn; Margarita, Dionysiou; Lysikatos, Charalampos; Belyavskaya, Elena; Chang, Richard; Stratakis, Constantine A

    2017-12-01

    The authors describe the clinical investigation of two cases of primary aldosteronism with adrenal hemorrhage (AH) following adrenal vein sampling. A literature review was conducted regarding the medical management of primary aldosteronism in patients with AH following adrenal vein sampling. Guidelines on the management of primary aldosteronism with AH following adrenal vein sampling are lacking. The two patients were followed with serial imaging to document resolution of AH and treated medically with excellent blood pressure response. Resolution of AH was achieved, but a repeat adrenal vein sampling was deferred given the increased morbidity risk associated with a repeat procedure. Published 2017. This article is a U.S. Government work and is in the public domain in the USA.

  16. [HPV infection associated with nonclassic cytological signs in patients from Mérida, Venezuela].

    Science.gov (United States)

    Méndez Toro, Luisana; Rodríguez Meza, Andry; López de Sánchez, Mercedes; Toro de Méndez, Morelva

    2011-06-01

    The purpose of this work was to identify those nonclassic cytological signs that would allow the prediction of human papillomavirus (HPV) infection. Cytology samples taken from outpatients that attended public and private gynecological practices, between January of 2006 and December of 2008, were studied. The cytological samples were informed following the criteria of the Bethesda system 2001. Those reports with a cytological interpretation of low-grade squamous intraepithelial lesion/HPV (LSIL/HPV) were selected. Age data and other cytologic characteristics were also taken. 25,565 Pap smears were revised, of which 291 were LSIL/HPV. The age average of the patients was 32.01 years and the age range was between 15 and 67 years. 55.10% of the patients were below 30 years of age. The koilocytosis was the most frequent viral cytological sign (66.1%). Among the HPV infection associated nonclassic cytologic signs were in order of frequency: nuclear enlargement (44%), megalocytosis (38.5%), followed by binucleation and lightly irregular chromatin distribution, in the same proportion (33.7%) and the typical parakeratotic cells (33.3%). Discreet nuclear hyperchromasia and hiper-keratotic cells were the less frequent morphological changes (28.5%). The indirect signs significantly associated to the infection were: binucleation, lightly irregular chromatin distribution, discreet nuclear hyperchromasia and para-keratotic cells. The combination of HPV infection associated with nonclassic cytological signs would allow the identification of a high proportion of patients with this infection, increasing in this way the sensibility of the cytological study in the detection of HPV and the possibility of selecting subgroups at risk for the development of cervical lesions and their opportune study.

  17. Cross-sectional observational study of 208 patients with non-classical urea cycle disorders.

    Science.gov (United States)

    Rüegger, Corinne M; Lindner, Martin; Ballhausen, Diana; Baumgartner, Matthias R; Beblo, Skadi; Das, Anibh; Gautschi, Matthias; Glahn, Esther M; Grünert, Sarah C; Hennermann, Julia; Hochuli, Michel; Huemer, Martina; Karall, Daniela; Kölker, Stefan; Lachmann, Robin H; Lotz-Havla, Amelie; Möslinger, Dorothea; Nuoffer, Jean-Marc; Plecko, Barbara; Rutsch, Frank; Santer, René; Spiekerkoetter, Ute; Staufner, Christian; Stricker, Tamar; Wijburg, Frits A; Williams, Monique; Burgard, Peter; Häberle, Johannes

    2014-01-01

    Urea cycle disorders (UCDs) are inherited disorders of ammonia detoxification often regarded as mainly of relevance to pediatricians. Based on an increasing number of case studies it has become obvious that a significant number of UCD patients are affected by their disease in a non-classical way: presenting outside the newborn period, following a mild course, presenting with unusual clinical features, or asymptomatic patients with only biochemical signs of a UCD. These patients are surviving into adolescence and adulthood, rendering this group of diseases clinically relevant to adult physicians as well as pediatricians. In preparation for an international workshop we collected data on all patients with non-classical UCDs treated by the participants in 20 European metabolic centres. Information was collected on a cohort of 208 patients 50% of which were ≥ 16 years old. The largest subgroup (121 patients) had X-linked ornithine transcarbamylase deficiency (OTCD) of whom 83 were female and 29% of these were asymptomatic. In index patients, there was a mean delay from first symptoms to diagnosis of 1.6 years. Cognitive impairment was present in 36% of all patients including female OTCD patients (in 31%) and those 41 patients identified presymptomatically following positive newborn screening (in 12%). In conclusion, UCD patients with non-classical clinical presentations require the interest and care of adult physicians and have a high risk of neurological complications. To improve the outcome of UCDs, a greater awareness by health professionals of the importance of hyperammonemia and UCDs, and ultimately avoidance of the still long delay to correctly diagnose the patients, is crucial.

  18. Non-classical effects of androgens on testes from neonatal rats.

    Science.gov (United States)

    da Rosa, Luciana Abreu; Escott, Gustavo Monteiro; Cavalari, Fernanda Carvalho; Schneider, Clara Maria Müller; de Fraga, Luciano Stürmer; Loss, Eloísa da Silveira

    2015-01-01

    The intratesticular testosterone concentration is high during the early postnatal period although the intracellular androgen receptor expression (iAR) is still absent in Sertoli cells (SCs). This study aimed to evaluate the non-classical effects of testosterone and epitestosterone on calcium uptake and the electrophysiological effects of testosterone (1μM) on SCs from rats on postnatal day (pnd) 3 and 4 with lack of expression of the iAR. In addition, crosstalk on the electrophysiological effects of testosterone and epitestosterone with follicle stimulating hormone (FSH) in SCs from 15-day-old rats was evaluated. The isotope (45)Ca(2+) was utilized to evaluate the effects of testosterone and epitestosterone in calcium uptake. The membrane potential of SCs was recorded using a standard single microelectrode technique. No immunoreaction concerning the iAR was observed in SCs on pnd 3 and 4. At this age, both testosterone and epitestosterone increased the (45)Ca(2+) uptake. Testosterone promoted membrane potential depolarization of SCs on pnd 4. FSH application followed by testosterone and epitestosterone reduced the depolarization of the two hormones. Application of epitestosterone 5 min after FSH resulted in a delay of epitestosterone-promoted depolarization. The cell resistance was also reduced. Thus, in SCs from neonatal Wistar rats, both testosterone and epitestosterone act through a non-classical mechanism stimulating calcium uptake in whole testes, and testosterone produces a depolarizing effect on SC membranes. Testosterone and epitestosterone stimulates non-classical actions via a membrane mechanism, which is independent of iAR. FSH and testosterone/epitestosterone affect each other's electrophysiological responses suggesting crosstalk between the intracellular signaling pathways. Copyright © 2014 Elsevier Inc. All rights reserved.

  19. Heat control in opto-mechanical system using quantum non-classicality

    International Nuclear Information System (INIS)

    Sharma, Sushamana; Senwar, Subash

    2016-01-01

    Cooling of matter to the quantum ground state is a primary directive of quantum control. In other words, to extract entropy from a quantum system, efficient indirect quantum measurements may be implemented. The main objective is the cooling of the oscillator either to its motional ground state or to non-classical states, such as low-number Fock states, squeezed states or entangled states. It is shown that the use of quantum control procedure is better choice for even experimental realizations because it leads to a squeezed steady state with less than one phonon on average. The steady state of system corresponds to cooling of the system.

  20. Oscillation frequency dependence of nonclassical rotation inertia of solid 4He.

    Science.gov (United States)

    Aoki, Y; Graves, J C; Kojima, H

    2007-07-06

    The nonclassical rotational inertia fraction of the identical cylindrical solid 4He below 300 mK is studied at 496 and 1173 Hz by a double resonance torsional oscillator. Below 35 mK, the fractions are the same at sufficiently low rim velocities. Above 35 mK, the fraction is greater for the higher than the lower mode. The dissipation peak of the lower mode occurs at a temperature approximately 4 mK lower than that of the higher mode. The drive dependence of the two modes shows that the reduction of the fraction is characterized by critical velocity, not amplitude or acceleration.

  1. Mammalian non-classical major histocompatibility complex I and its receptors: Important contexts of gene, evolution, and immunity

    Science.gov (United States)

    Pratheek, B. M.; Nayak, Tapas K.; Sahoo, Subhransu S.; Mohanty, Prafulla K.; Chattopadhyay, Soma; Chakraborty, Ntiya G.; Chattopadhyay, Subhasis

    2014-01-01

    The evolutionary conserved, less-polymorphic, nonclassical major histocompatibility complex (MHC) class I molecules: Qa-1 and its human homologue human leukocyte antigen-E (HLA-E) along with HLA-F, G and H cross-talk with the T-cell receptors and also interact with natural killer T-cells and other lymphocytes. Moreover, these nonclassical MHC molecules are known to interact with CD94/NKG2 heterodimeric receptors to induce immune responses and immune regulations. This dual role of Qa-1/HLA-E in terms of innate and adaptive immunity makes them more interesting. This review highlights the new updates of the mammalian nonclassical MHC-I molecules in terms of their gene organization, evolutionary perspective and their role in immunity. PMID:25400340

  2. Expression of bovine non-classical major histocompatibility complex class 1 proteins in mouse P815 and human K562 cells

    Science.gov (United States)

    Major histocompatibility complex class I (MHC-I) proteins can be expressed as cell surface or secreted proteins. To investigate whether bovine non-classical MHC-I proteins are expressed as cell surface or secreted proteins, and to assess the reactivity pattern of monoclonal antibodies with non-class...

  3. Microwave thermotherapy for benign prostatic hyperplasia.

    Science.gov (United States)

    Hoffman, Richard M; Monga, Manoj; Elliott, Sean P; Macdonald, Roderick; Langsjoen, Jens; Tacklind, James; Wilt, Timothy J

    2012-09-12

    Transurethral resection of the prostate (TURP) has been the gold-standard treatment for alleviating urinary symptoms and improving urinary flow in men with symptomatic benign prostatic hyperplasia (BPH). However, the morbidity of TURP approaches 20%, and less invasive techniques have been developed for treating BPH. Preliminary data suggest that microwave thermotherapy, which delivers microwave energy to produce coagulation necrosis in prostatic tissue, is a safe, effective treatment for BPH. To assess the therapeutic efficacy and safety of microwave thermotherapy techniques for treating men with symptomatic benign prostatic obstruction. Randomized controlled trials were identified from The Cochrane Library, MEDLINE, EMBASE, bibliographies of retrieved articles, reviews, technical reports, and by contacting relevant expert trialists and microwave manufacturers. All randomized controlled trials evaluating transurethral microwave thermotherapy (TUMT) for men with symptomatic BPH were eligible for this review. Comparison groups could include transurethral resection of the prostate, minimally invasive prostatectomy techniques, sham thermotherapy procedures, and medications. Outcome measures included urinary symptoms, urinary function, prostate volume, mortality, morbidity, and retreatment. Two review authors independently identified potentially relevant abstracts and then assessed the full papers for inclusion. Two review authors independently abstracted study design, baseline characteristics, and outcomes data and assessed methodological quality using a standard form. We attempted to obtain missing data from authors or sponsors, or both. In this update, we identified no new randomized comparisons of TUMT that provided evaluable effectiveness data. Fifteen studies involving 1585 patients met the inclusion criteria, including six comparisons of microwave thermotherapy with TURP, eight comparisons with sham thermotherapy procedures, and one comparison with an alpha

  4. Spatial-Temporal Expression of Non-classical MHC Class I Molecules in the C57 Mouse Brain.

    Science.gov (United States)

    Liu, Jiane; Shen, Yuqing; Li, Mingli; Lv, Dan; Zhang, Aifeng; Peng, Yaqin; Miao, Fengqin; Zhang, Jianqiong

    2015-07-01

    Recent studies clearly demonstrate major histocompatibility complex (MHC) class I expression in the brain plays an important functional role in neural development and plasticity. A previous study from our laboratory demonstrated the temporal and spatial expression patterns of classical MHC class I molecules in the brain of C57 mice. Studies regarding non-classical MHC class I molecules remain limited. Here we examine the expression of non-classical MHC class I molecules in mouse central nervous system (CNS) during embryonic and postnatal developmental stages using in situ hybridization and immunofluorescence. We find non-classical MHC class I molecules, M3/T22/Q1, are expressed in the cerebral cortex, neuroepithelium of the lateral ventricle, neuroepithelium of aquaeductus and developing cerebellum during embryonic developmental stages. During the postnatal period from P0 to adult, non-classical MHC class I mRNAs are detected in olfactory bulb, hippocampus, cerebellum and some nerve nuclei. Overall, the expression patterns of non-classical MHC class I molecules are similar to those of classical MHC class I molecules in the developing mouse brain. In addition, non-classical MHC class I molecules are present in the H2-K(b) and H2-D(b) double knock-out mice where their expression levels are greatly increased within the same locations as compared to wild type mice. The elucidation and discovery of the expression profile of MHC class I molecules during development is important for supporting an enhanced understanding of their physiological and potential pathological roles within the CNS.

  5. SPRED: A machine learning approach for the identification of classical and non-classical secretory proteins in mammalian genomes

    International Nuclear Information System (INIS)

    Kandaswamy, Krishna Kumar; Pugalenthi, Ganesan; Hartmann, Enno; Kalies, Kai-Uwe; Moeller, Steffen; Suganthan, P.N.; Martinetz, Thomas

    2010-01-01

    Eukaryotic protein secretion generally occurs via the classical secretory pathway that traverses the ER and Golgi apparatus. Secreted proteins usually contain a signal sequence with all the essential information required to target them for secretion. However, some proteins like fibroblast growth factors (FGF-1, FGF-2), interleukins (IL-1 alpha, IL-1 beta), galectins and thioredoxin are exported by an alternative pathway. This is known as leaderless or non-classical secretion and works without a signal sequence. Most computational methods for the identification of secretory proteins use the signal peptide as indicator and are therefore not able to identify substrates of non-classical secretion. In this work, we report a random forest method, SPRED, to identify secretory proteins from protein sequences irrespective of N-terminal signal peptides, thus allowing also correct classification of non-classical secretory proteins. Training was performed on a dataset containing 600 extracellular proteins and 600 cytoplasmic and/or nuclear proteins. The algorithm was tested on 180 extracellular proteins and 1380 cytoplasmic and/or nuclear proteins. We obtained 85.92% accuracy from training and 82.18% accuracy from testing. Since SPRED does not use N-terminal signals, it can detect non-classical secreted proteins by filtering those secreted proteins with an N-terminal signal by using SignalP. SPRED predicted 15 out of 19 experimentally verified non-classical secretory proteins. By scanning the entire human proteome we identified 566 protein sequences potentially undergoing non-classical secretion. The dataset and standalone version of the SPRED software is available at (http://www.inb.uni-luebeck.de/tools-demos/spred/spred).

  6. Approach to the handling of adrenal insufficiency Manejo de la insuficiencia adrenal

    Directory of Open Access Journals (Sweden)

    Federico Uribe Londoño

    2005-03-01

    Full Text Available The term adrenal insufficiency refers to the hypofunction of this gland. From the etiologic point of view it may be either primary or secondary. This insufficiency manifests as inadequate serum levels of cortisol and adrenal androgens in the secondary form and of these and mineralocorticoids in the primary one. Clinical manifestations are often nonspecific and, consequently, diagnosis may be difficult to establish and treatment may be delayed with increased morbidity and mortality. This article on adrenal insufficiency includes its definition, epidemiology, etiology, physiopathology, classification, clinical presentation, diagnostic criteria and treatment guidelines. Besides, some special situations like critically ill patients and pregnant women are given special consideration. Emphasis is done on a diagnostic algorithm to make it easier for general practitioners the approach to patients with this endocrine disorder. La insuficiencia adrenal (ia se refiere a la hipofunción de dicha glándula debida a causas tanto primarias como secundarias, que resultan en niveles plasmáticos inadecuados de cortisol, andrógenos adrenales y adicionalmente, en la falla primaria de mineralocorticoides. Sus manifestaciones inespecíficas dificultan o retrasan con frecuencia el diagnóstico y tratamiento oportuno, lo cual incrementa la morbilidad y eventualmente la mortalidad de estos pacientes. Se incluyen en este artículo: definición, epidemiología, causas, fisiopatología, clasificación, manifestaciones clínicas, diagnóstico y tratamiento de la IA. Además se consideran algunas situaciones especiales como la IA en el paciente críticamente enfermo y en la mujer gestante. Finalmente se hace especial énfasis en un algoritmo diagnóstico, con la finalidad de facilitarle al médico general un enfoque ágil y oportuno de los pacientes con este problema endocrino.

  7. Adrenal gland infection by serotype 5 adenovirus requires coagulation factors.

    Directory of Open Access Journals (Sweden)

    Lucile Tran

    Full Text Available Recombinant, replication-deficient serotype 5 adenovirus infects the liver upon in vivo, systemic injection in rodents. This infection requires the binding of factor X to the capsid of this adenovirus. Another organ, the adrenal gland is also infected upon systemic administration of Ad, however, whether this infection is dependent on the cocksackie adenovirus receptor (CAR or depends on the binding of factor X to the viral capsid remained to be determined. In the present work, we have used a pharmacological agent (warfarin as well as recombinant adenoviruses lacking the binding site of Factor X to elucidate this mechanism in mice. We demonstrate that, as observed in the liver, adenovirus infection of the adrenal glands in vivo requires Factor X. Considering that the level of transduction of the adrenal glands is well-below that of the liver and that capsid-modified adenoviruses are unlikely to selectively infect the adrenal glands, we have used single-photon emission computed tomography (SPECT imaging of gene expression to determine whether local virus administration (direct injection in the kidney could increase gene transfer to the adrenal glands. We demonstrate that direct injection of the virus in the kidney increases gene transfer in the adrenal gland but liver transduction remains important. These observations strongly suggest that serotype 5 adenovirus uses a similar mechanism to infect liver and adrenal gland and that selective transgene expression in the latter is more likely to be achieved through transcriptional targeting.

  8. Giant Benign Prostatic Hyperplasia in a Pakistani Patient

    Directory of Open Access Journals (Sweden)

    Zafaruddin Khan

    2014-01-01

    Full Text Available “Giant hyperplasia” of the prostate is a rare pathology of the prostate gland. We report one such case, in which a successful retropubic prostatectomy was performed on an elderly male patient in Pakistan. The weight of the resected prostate was 700 g, which is the eighth largest prostate with benign prostatic hyperplasia reported.

  9. An Audit of Endometrial Hyperplasias at the Lagos University ...

    African Journals Online (AJOL)

    2017-10-26

    Oct 26, 2017 ... Introduction: There has been much controversy and confusion surrounding the endometrial hyperplasias stemming from the use of a wide variety of terminologies and also from the pathophysiologic mechanisms underlying the various entities. The current classification by the World Health Organization ...

  10. Pituitary hyperplasia secondary to hypothyroidism in an adolescent

    International Nuclear Information System (INIS)

    Capiel, Carlos A. h; Bouzas, Carlos A.; Mondino, Ana

    2003-01-01

    We report a case of a 14 years old patient with growth arrest and laboratory evidence of hypothyroidism. MR revealed pituitary enlargement simulating macro adenoma. Thyroid replacement therapy resulted in regression of the pituitary size. Awareness of MR appearance of pituitary hyperplasia in children and juvenile patients with laboratory evidence of hypothyroidism might avoid misdiagnosis for pituitary tumor. (author)

  11. Two Cases of. Cushing's Syndrome tumour and bilateral hyperplasia

    African Journals Online (AJOL)

    Two patients, one with Cushing's syndrome and one with Cushing's disease, are presented. In the first case the syndrome was caused by a tumour of the right suprarenal gland which was treated by unilateral adrenalectomy, and the second case was diagnosed as hyperplasia of the left suprarenal gland, eventually leading ...

  12. Gingival hyperplasia induced by diphenylhydantoin in a gorilla.

    Science.gov (United States)

    Fagan, D; Oosterhuis, J

    1979-11-01

    An adult male lowland gorilla had been treated with diphenylhydantoin for 6 months following several acute convulsive episodes. The gorilla remained clinically normal during that period. Then, for no apparent reason, it refused its usual diet. Physical examination revealed acute inflammatory gingival hyperplasia. Full mouth gingivectomy and antibiotic and analgesic therapy resolved the oral inflammation and the anorexia.

  13. Update in minimal invasive therapy in benign prostatic hyperplasia.

    NARCIS (Netherlands)

    Hest, P.J. van; D'Ancona, F.C.H.

    2009-01-01

    Last decade several new treatment modalities for minimal invasive therapy of benign prostatic hyperplasia have been developed, both ablative and non-ablative. In this review the authors describe the different techniques and clinical studies of bipolar transurethral resection of the prostate (TURP),

  14. A mouse model of mammary hyperplasia induced by oral hormone ...

    African Journals Online (AJOL)

    Methods and Materials: To address the mechanism, we developed a mouse model of mammary hyperplasia. We gave mice estradiol valerate tablets and progesterone capsules sequentially for one month by intragastric administration. Results: Mice treated by this method had a series of pathological changes which are ...

  15. Effect of Prunella vulgaris L extract on hyperplasia of mammary ...

    African Journals Online (AJOL)

    p < 0.01) in rats treated with highdose PVE. Conclusion: These results suggest that PVE exerts anti-HMG effect in rats induced by estrogen and progestogen. Keywords: Prunella vulgaris L; Anti-inflammatory; Anti-hyperplasia of mammary gland ...

  16. Management of experimental benign prostatic hyperplasia in rats ...

    African Journals Online (AJOL)

    The usefulness of diet containing Telfairia occidentalis seeds, in managing benign prostatic hyperplasia (BPH) in rats was studied. Twenty male Wistar rats were divided into four equal groups. BPH was induced by sub-cutaneous injection of dihydrotestosterone (DHT) and estradiol valerate (ratio, 10:1) every other day for ...

  17. Evaluation of Ovarian Lesions Inducing Endometrial Hyperplasia or ...

    African Journals Online (AJOL)

    Mubeen

    Background: Excessive and prolonged estrogenic stimulation results in endometrial hyperplasias or endometrioid adenocarcinomas. One of the major reasons for an excess endogenous estrogen production is estrogen secreting ovarian lesions which could either be neoplastic or non-neoplastic. Aims: This was a study ...

  18. Angiolymphoid Hyperplasia with Eosinophilia of Orbit in Young Male

    African Journals Online (AJOL)

    [3] The etiology of. Angiolymphoid Hyperplasia with Eosinophilia of Orbit in Young Male. Somen Misra, Akshay Bhandari, Sagar Chaudhari, Neeta Misra, Pratik Gogri, Parag Tupe. Department of Ophthalmology, Rural Medical .... blood eosinophilia, and nephrotic syndrome due to IgE deposition in the renal glomeruli.

  19. Qianliening capsule treats benign prostatic hyperplasia (BPH) by ...

    African Journals Online (AJOL)

    Benign prostatic hyperplasia (BPH) is characterized by abnormal proliferation of epithelial and stromal cells in prostatic tissue, which is closely correlated with increased expression of PCNA, CyclinD1 and CDK4. Therefore, inhibition of cell proliferation by suppressing the expression of the above genes is a promising ...

  20. Evaluation of Ovarian Lesions Inducing Endometrial Hyperplasia or ...

    African Journals Online (AJOL)

    Conclusion: Follicular cyst was the predominant lesion associated with endometrial hyperplasia and endometrioid adenocarcinoma of endometrium in post-menopausal age group. In addition, hyperplastic lesions in endometrium can occur in non-hormonal secreting ovarian epithelial tumors possibly due to functioning ...