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

Growth and development in children with classic congenital adrenal hyperplasia.

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Bonfig, Walter

2017-02-01

Final height outcome in classic congenital adrenal hyperplasia (CAH) has been of interest for many years. With analysis of growth patterns and used glucocorticoid regimens, enhanced treatment strategies have been developed and are still under development. Most of the current reports on final height outcome are confirmative of previous results. Final height data is still reported in cohorts that were diagnosed clinically and not by newborn screening. Clinical diagnosis of CAH leads to delayed diagnosis especially of simple virilizing CAH with significantly advanced bone age resulting in early pubertal development and reduced final height. In contrast salt-wasting CAH is diagnosed at an earlier stage in most cases resulting in better final height outcome in some cohorts. Nevertheless, final height outcome in patients with CAH treated with glucocorticoids is lower than the population norm and also at the lower end of genetic potential. Achievement of regular adult height is still a challenge with conventional glucocorticoid treatment in patients with CAH, which is why new hydrocortisone formulations and new treatment options for CAH are underway.

Nonclassic Congenital Adrenal Hyperplasia

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Selma Feldman Witchel

2010-01-01

Full Text Available Nonclassic congenital adrenal hyperplasia (NCAH due to P450c21 (21-hydroxylase deficiency is a common autosomal recessive disorder. This disorder is due to mutations in the CYP21A2 gene which is located at chromosome 6p21. The clinical features predominantly reflect androgen excess rather than adrenal insufficiency leading to an ascertainment bias favoring diagnosis in females. Treatment goals include normal linear growth velocity and “on-time” puberty in affected children. For adolescent and adult women, treatment goals include regularization of menses, prevention of progression of hirsutism, and fertility. This paper will review key aspects regarding pathophysiology, diagnosis, and treatment of NCAH.

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.

Aldosterone hypersecretion in “non-salt-losing” congenital adrenal hyperplasia

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Bartter, Frederic C.; Henkin, Robert I.; Bryan, George T.

1968-01-01

Patients with the “non-salt-losing” form of the adrenogenital syndrome were studied before and after suppression of adrenal cortical activity with carbohydrate-active steroids. The response of aldosterone secretion to sodium deprivation was measured; in some patients response to adrenocorticotropic hormone (ACTH) was measured as well. The aldosterone secretion was normal and responded normally to sodium deprivation in all patients studied during suppression with carbohydrate-active steroids. This finding suggests that 21-hydroxylation of progesterone is normal in this syndrome. The sole abnormality in the production of aldosterone in these patients was found to be excessive secretion of aldosterone while they were not receiving suppressive doses of carbohydrate-active steroids. This finding strongly supports the view that the biogenetic pathways through which aldosterone is produced from progesterone are intact in this syndrome. No patient showed hypertension or hypokalemic alkalosis despite very high aldosterone secretion rates. This observation suggests that the hyper-aldosteronism is secondary to a tendency to sodium loss in the patient whose ACTH production is not suppressed. These studies provide additional evidence in support of the hypothesis that the salt-losing and “non-salt-losing” forms of adrenogenital syndrome are genetically and biochemically distinct. PMID:4299011

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.

Ultrasound follow up of testicular adrenal rest tumors with congenital adrenal hyperplasia: Report of three cases

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Cho, Jeong Yeon; Kim, Dong Won; Yoon, Seong Kuk; Nam, Kyung Jin [Dept. of Radiology, Dong-A University Hospital, Busan (Korea, Republic of)

2014-12-15

While testicular adrenal rest tumor is generally a rare intratesticular tumor, it is frequent in patients with congenital adrenal hyperplasia. The tumors are diagnosed and followed up by ultrasound examination because these tumors are non-palpable and symptomless in most cases and always benign. Ultrasound imaging features change depending on how congenital adrenal hyperplasia is controlled. We herein report three cases of testicular adrenal rest tumors with different usual and unusual imaging findings and follow-up imaging. Patient 1 was a 14-year-old boy who presented with poor compliance to medication. Patient 2 and 3 were a 10-year-old and 13-year-old boy who presented with precocious puberty and short stature, respectively. Ultrasound examinations demonstrated oval hypoechoic masses and irregular speculated hyperechoic masses in the testes and different serial imaging findings.

Ultrasound follow up of testicular adrenal rest tumors with congenital adrenal hyperplasia: Report of three cases

International Nuclear Information System (INIS)

Cho, Jeong Yeon; Kim, Dong Won; Yoon, Seong Kuk; Nam, Kyung Jin

2014-01-01

While testicular adrenal rest tumor is generally a rare intratesticular tumor, it is frequent in patients with congenital adrenal hyperplasia. The tumors are diagnosed and followed up by ultrasound examination because these tumors are non-palpable and symptomless in most cases and always benign. Ultrasound imaging features change depending on how congenital adrenal hyperplasia is controlled. We herein report three cases of testicular adrenal rest tumors with different usual and unusual imaging findings and follow-up imaging. Patient 1 was a 14-year-old boy who presented with poor compliance to medication. Patient 2 and 3 were a 10-year-old and 13-year-old boy who presented with precocious puberty and short stature, respectively. Ultrasound examinations demonstrated oval hypoechoic masses and irregular speculated hyperechoic masses in the testes and different serial imaging findings

Radiological findings of congenital lipoid adrenal hyperplasia: a case report

International Nuclear Information System (INIS)

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

Prenatal Diagnosis of Congenital Adrenal Hyperplasia.

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Yau, Mabel; Khattab, Ahmed; New, Maria I

2016-06-01

Congenital adrenal hyperplasia (CAH) owing to 21-hydroxylase deficiency is a monogenic disorder of adrenal steroidogenesis. To prevent genital ambiguity, in girls, prenatal dexamethasone treatment is administered early in the first trimester. Prenatal genetic diagnosis of CAH and fetal sex determination identify affected female fetuses at risk for genital virilization. Advancements in prenatal diagnosis are owing to improved understanding of the genetic basis of CAH and improved technology. Cloning of the CYP21A2 gene ushered in molecular genetic analysis as the current standard of care. Noninvasive prenatal diagnosis allows for targeted treatment and avoids unnecessary treatment of males and unaffected females. Copyright © 2016 Elsevier Inc. All rights reserved.

Classic congenital adrenal hyperplasia and puberty.

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Charmandari, Evangelia; Brook, Charles G D; Hindmarsh, Peter C

2004-11-01

Congenital adrenal hyperplasia (CAH) is a group of autosomal recessive disorders resulting from deficiency of one of the five enzymes required for synthesis of cortisol in the adrenal cortex. The most common form of the disease is classic 21-hydroxylase deficiency, which is characterized by decreased synthesis of glucocorticoids and often mineralocorticoids, adrenal hyperandrogenism and impaired development and function of the adrenal medulla. The clinical management of classic 21-hydroxylase deficiency is often suboptimal, and patients are at risk of developing in tandem iatrogenic hypercortisolism and/or hyperandogenism. Limitations of current medical therapy include the inability to control hyperandrogenism without employing supraphysiologic doses of glucocorticoid, hyperresponsiveness of the hypertrophied adrenal glands to adrenocorticotropic hormone (ACTH) and difficulty in suppressing ACTH secretion from the anterior pituitary. Puberty imposes increased difficulty in attaining adrenocortical suppression despite optimal substitution therapy and adherence to medical treatment. Alterations in the endocrine milieu at puberty may influence cortisol pharmacokinetics and, consequently, the handling of hydrocortisone used as replacement therapy. Recent studies have demonstrated a significant increase in cortisol clearance at puberty and a shorter half-life of free cortisol in pubertal females compared with males. Furthermore, children with classic CAH have elevated fasting serum insulin concentrations and insulin resistance. The latter may further enhance adrenal and/or ovarian androgen secretion, decrease the therapeutic efficacy of glucocorticoids and contribute to later development of the metabolic syndrome and its complications.

Pitfalls in molecular diagnosis of 21-hydroxylase deficiency in congenital adrenal hyperplasia.

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Kolahdouz, Mahsa; Mohammadi, Zahra; Kolahdouz, Parisa; Tajamolian, Masoud; Khanahmad, Hossein

2015-01-01

Congenital adrenal hyperplasia (CAH) is a putative error of metabolism with autosomal recessive heredity pattern. The main manifestations of classic form of CAH are salt-wasting, dehydration and simple virilization in both sexes and ambiguous genitalia in female gender. 21-hyroxylase (CYP21A2) impairment with prevalence value of 1 in 10,000-15,000 live births is the most common etiology of CAH. Because of consanguineous marriages, the frequency of the CAH in Iran is very high. A wide range of mutations diversity exists in CYP21A2 gene and a large number of these mutations derived from a highly homologous pseudogene, CYP21A1P, through gene conversion. In addition, new mutations such as small and large deletion and point mutations can also result in enzyme deficiency. Various methods for mutation detection were performed. The main obstacle in molecular diagnosis of CAH is amplification of pseudogene during polymerase chain reaction of CYP21A2. All attempts focus on discrimination of pseudogene from gene; that is why, there is the majority of mutations on pseudogene, and if we have contamination with the pseudogene, the result will be unreliable. Here, we discuss this methods and advantage and disadvantage of those.

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

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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.

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

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Ruiz-Babot, Gerard; Balyura, Mariya; Hadjidemetriou, Irene; Ajodha, Sharon J; Taylor, David R; Ghataore, Lea; Taylor, Norman F; Schubert, Undine; Ziegler, Christian G; Storr, Helen L; Druce, Maralyn R; Gevers, Evelien F; Drake, William M; Srirangalingam, Umasuthan; Conway, Gerard S; King, Peter J; Metherell, Louise A; Bornstein, Stefan R; Guasti, Leonardo

2018-01-30

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. Copyright © 2018 The Authors. Published by Elsevier Inc. All rights reserved.

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

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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.

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.

Reconstructive surgery for females with congenital adrenal hyperplasia due to 21-hydroxylase deficiency: a review from the Prince of Wales Hospital.

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Houben, C H; Tsui, S Y; Mou, J W; Chan, K W; Tam, Y H; Lee, K H

2014-12-01

To present the results of feminising genitoplasty done in female patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Case series. A tertiary referral centre in Hong Kong. Female patients with congenital adrenal hyperplasia undergoing corrective surgery for virilisation between 1993 and 2012. The operative result was judged with a scoring system (1-3) for four areas: appearance of clitoris, labia and vagina, plus requirement for revision surgery. A total of 23 female patients with congenital adrenal hyperplasia with a median age of 17.5 (range, 1.5-33.8) years were identified. Of these individuals, 17 presented in the neonatal period and early infancy, of which four had an additional salt-losing crisis. Six patients-including four migrants from mainland China-were late presenters at a median age of 2 (range, 0.5-14) years. Twenty-two patients had corrective surgery at a median age of 2 (range, 1-14) years. Clitoral reduction was performed in all, and further surgery in 21 patients. The additional surgery was flap vaginoplasty in 10 patients, a modified Passerini procedure in six, and a labial reconstruction in five; one patient with prominent clitoris was for observation only. Minor revision surgery (eg mucosal trimming) was required in three patients; a revision vaginoplasty was done in one individual. Of the 23 patients, 18 (78%) with a median age of 20 (range, 9.3-33.8) years participated in the outcome evaluation: a 'good' outcome (4 points) was seen in 12 patients and a 'satisfactory' (5-9 points) result in five patients. Nearly three quarters of our cohort (n=17) presented with classic virilising form of 21-hydroxylase deficiency. Only four (25%) patients experienced a salt-losing crisis. Female gender assignment at birth was maintained for all individuals in this group. 'Good' and 'satisfactory' outcomes of surgery were reported in nearly all participants.

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.

2014-01-01

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

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

NARCIS (Netherlands)

Stikkelbroeck, N.M.; Hermus, A.R.M.M.; Schouten, D.; Suliman, H.M.; Jager, G.J.; Braat, D.D.M.; Otten, B.J.

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

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

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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

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

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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 (...

Congenital Adrenal Hyperplasia: Unresolved Issues.

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Yau, Mabel; Khattab, Ahmed; Poppas, Dix; Ghizzoni, Lucia; New, Maria

2016-01-01

Congenital adrenal hyperplasia (CAH) describes a family of disorders that comes from enzymatic deficiencies in cortisol production, with 21-hydroxylase deficiency causing ∼90% of cases. Distinction is made between the severe classical form and milder nonclassical form of CAH. Molecular genetic analysis is used to confirm the hormonal diagnosis. A high rate of genotype-phenotype disconcordance has been found in 21-hydroxylase deficiency. The goal of treatment is to replace with synthetic glucocorticoids and mineralocorticoids and suppress adrenal androgen production. The treatment of patients affected with nonclassical CAH, particularly males, remains controversial. Variable synthetic glucocorticoids are used and new modes of glucocorticoid delivery are under investigation. To improve height, growth hormone and other adjuvant therapies are employed. Long-term outcomes of genital surgery using modern techniques in females affected with classical CAH continue to be investigated. Prenatal treatment with dexamethasone is available to avoid ambiguous genitalia in these females. Although studies have shown its safety to mother and fetus, prenatal treatment is still regarded as experimental. Currently, prenatal diagnosis of CAH can only be obtained through invasive methods. Recently, the detection of cell-free fetal DNA in maternal plasma has made it possible to make this diagnosis earlier and noninvasively. © 2016 S. Karger AG, Basel.

Unusual phenotype of congenital adrenal hyperplasia (CAH) with a novel mutation of the CYP21A2 gene.

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Raisingani, Manish; Contreras, Maria F; Prasad, Kris; Pappas, John G; Kluge, Michelle L; Shah, Bina; David, Raphael

2016-07-01

Gonadotropin independent sexual precocity (SP) may be due to congenital adrenal hyperplasia (CAH), and its timing usually depends on the type of mutation in the CYP21A2 gene. Compound heterozygotes are common and express phenotypes of varying severity. The objective of this case report was to investigate the hormonal pattern and unusual genetic profile in a 7-year-old boy who presented with pubic hair, acne, an enlarged phallus, slightly increased testicular volume and advanced bone age. Clinical, hormonal and genetic studies were undertaken in the patient as well as his parents. We found elevated serum 17-hydroxyprogesterone (17-OHP) and androstenedione that were suppressed with dexamethasone, and elevated testosterone that actually rose after giving dexamethasone, indicating activity of the hypothalamic-pituitary-gonadal (HPG) axis. An initial search for common mutations was negative, but a more detailed genetic analysis of the CYP21A2 gene revealed two mutations including R341W, a non-classical mutation inherited from his mother, and g.823G>A, a novel not previously reported consensus donor splice site mutation inherited from his father, which is predicted to be salt wasting. However, the child had a normal plasma renin activity. He was effectively treated with low-dose dexamethasone and a GnRH agonist. His father was an unaffected carrier, but his mother had evidence of mild non-classical CAH. In a male child presenting with gonadotropin independent SP it is important to investigate adrenal function with respect to the androgen profile, and to carry out appropriate genetic studies.

A three-year follow-up of congenital adrenal hyperplasia newborn screening.

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Pezzuti, Isabela L; Barra, Cristina B; Mantovani, Rafael M; Januário, José N; Silva, Ivani N

2014-01-01

congenital adrenal hyperplasia (CAH) newborn screening can prevent neonatal mortality in children with the salt-wasting form of the disease and prevent incorrect gender assignments, which can occur in females. However, the occurrence of false-positive results in preterm or low-birth-weight newborns creates some diagnostic difficulties, with consequent therapeutic implications. This study aimed to report the results of a pilot project for neonatal CAH screening conducted in the state of Minas Gerais, Brazil from 09/2007 to 05/2008 with a three-year follow-up. dried blood specimens were collected on filter paper cards three to seven days after birth of all newborns in the period. Samples were analyzed for 17-hydroxyprogesterone using an enzyme-linked immunosorbent assay (ELISA). a total of 159,415 children were screened. The apparent incidence of the classic variant of the disease was 1:9,963, based on initial diagnoses following newborn screening. During the follow-up period, eight of 16 children initially diagnosed with CAH were reclassified as unaffected, resulting in a revised incidence of 1:19,927. The false-positive rate was 0.31%, and the positive predictive value was 2.1%. Sensitivity and specificity were 100% and 99.7%, respectively. newborn screening is an important public health policy in developing countries such as Brazil, where CAH remains underdiagnosed. It has great potential to identify children with the disease who otherwise cannot be diagnosed earlier. Long-term follow-up and monitoring of all children with positive screening results are crucial to ensure a correct diagnosis and to calculate a reliable incidence ratio of the disease. Copyright © 2014 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.

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

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...

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

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Yaser Ramírez Benítez

2008-12-01

brain damage by pathologic effect of sexual hormones in developing nervous system, and also, are the secondary sequelae to severe situation of adrenal crises appearing in a classic way in the first days of life. METHODS: a study-case of a child aged 6 presenting with backgrounds of congenital adrenal hyperplasia and salt-losing syndrome. We determine weak and strong points in develop by means of the Luria Inicial neuropsychological battery. As a complement to valuation we applied the Weschler Intelligence Scale and the Infantile Behavior Questionnaire for parents and professors. RESULTS: child presenting with a neuropsychological profile characterized by low scores in: manual motility, visual-spatial, impulsiveness, a poor language, difficulty in operations of simple calculations, in recognition of objects by touch with visual help, and in processing speed. Ability of intelligence is within normal limits with the lower performance in non-verbal scale. In behavior scale, it was possible to identify difficulties to attention and learning. CONCLUSION: neuropsychological profile is characterized by a low level of executions in verbal tasks, non-verbal, and in processing speed. Hormonal treatment during first days of life allows that development follows its "normal" course, but all process has a cost reflected in acquisition of higher psychological abilities

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

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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…

Feminising genitoplasty: one-stage genital reconstruction in congenital adrenal hyperplasia: 30 years' experience.

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Roll, M F; Kneppo, C; Roth, H; Bettendorf, M; Waag, K-L; Holland-Cunz, S

2006-10-01

The study objective is to evaluate the results of our surgical technique for children with congenital adrenal hyperplasia and ambiguous genitalia at the University Hospital of Heidelberg, Department of Paediatric Surgery. The records of 19 patients with congenital adrenal hyperplasia treated between 1972 and 2004 were reviewed with respect to age at surgery, operative procedures and outcome. We describe the recession clitoroplasty technique currently used in our hospital and highlight the importance of short and long-term follow-up results with respect to appearance, position and size of the clitoris and quality of the vagina. One-stage recession clitoroplasty and vaginoplasty gives very satisfactory cosmetic and functional results, with few complications and a reduced need for secondary surgical interventions. The results of this study support the assumption that total correction can be achieved through a single-stage operation, performed in infancy.

Congenital Adrenal Hyperplasia

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Congenital adrenal hyperplasia with localized aggressive periodontitis and amelogenesis imperfecta.

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Ajlan, Sumaiah Abdulbaqi

2015-11-01

Congenital adrenal hyperplasia (CAH) is an inherited medical condition that implies defects in steroid biosynthesis. The dental findings of a female patient with CAH are reported. The patient suffered from severe periodontal tissue destruction, obvious enamel defects, as well as some occlusal problems. The management approach is presented and the possibility of interrelation of her dental findings with her medical condition is discussed. © 2015 Japanese Teratology Society.

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

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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.

A rare CYP21A2 mutation in a congenital adrenal hyperplasia kindred displaying genotype-phenotype nonconcordance.

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Khattab, Ahmed; Yuen, Tony; Al-Malki, Sultan; Yau, Mabel; Kazmi, Diya; Sun, Li; Harbison, Madeleine; Haider, Shozeb; Zaidi, Mone; New, Maria I

2016-01-01

Congenital adrenal hyperplasia (CAH) owing to 21-hydroxylase deficiency is caused by the autosomal recessive inheritance of mutations in the gene CYP21A2. CYP21A2 mutations lead to variable impairment of the 21-hydroxylase enzyme, which, in turn, is associated with three clinical phenotypes, namely, salt wasting, simple virilizing, and nonclassical CAH. However, it is known that a given mutation can associate with different clinical phenotypes, resulting in a high rate of genotype-phenotype nonconcordance. We aimed to study the genotype-phenotype nonconcordance in a family with three siblings affected with nonclassical CAH. All had hormonal evidence of nonclassical CAH, but this phenotype could not be explained by the genotype obtained from commercial CYP21A2 genetic testing, which revealed heterozygosity for the maternal 30 kb deletion mutation. We performed Sanger sequencing of the entire CYP21A2 gene in this family to search for a rare mutation that was not covered by commercial testing and found in the three siblings a second, rare c.1097G>A (p.R366H) mutation in exon 8. Computational modeling confirmed that this was a mild mutation consistent with nonclassical CAH. We recommend that sequencing of entire genes for rare mutations should be carried out when genotype-phenotype nonconcordance is observed in patients with autosomal recessive monogenic disorders, including CAH. © 2015 New York Academy of Sciences.

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...

Should male gender assignment be considered in the markedly virilized patient With 46,XX and congenital adrenal hyperplasia?

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Lee, Peter A; Houk, Christopher P; Husmann, Douglas A

2010-10-01

We assess the outcome in 46,XX men with congenital adrenal hyperplasia who were born with Prader 4 or 5 genitalia and assigned male gender at birth. After receiving institutional review board approval and subject consent we reviewed the medical records of 12 men 35 to 69 years old with 46,XX congenital adrenal hyperplasia, of whom 6 completed social and gender issue questionnaires. All subjects were assigned male gender at birth, were diagnosed with virilizing congenital adrenal hyperplasia at age greater than 3 years and indicated a male gender identity with sexual orientation to females. Ten of the 12 subjects had always lived as male and 2 who were reassigned to female gender in childhood subsequently self-reassigned as male. Nine of the 12 men had long-term female partners, including 7 married 12 years or more. The 3 subjects without a long-term female partner included 1 priest, 1 who was reassigned female gender, married, divorced and self-reassigned as male, and 1 with a girlfriend and sexual activity. All except the priest and the subject who was previously married when female indicated a strong libido and frequent orgasmic sexual activity. Responses to self-esteem, masculinity, body image, social adjustment and symptom questionnaires suggested adjustments related to the extent of familial and social support. Outcome data on severely masculinized 46,XX patients with congenital adrenal hyperplasia who were assigned male gender at birth indicate male gender identity in adulthood with satisfactory male sexual function in those retaining male genitalia. In men who completed questionnaires results were poorer in those lacking familial/social support. Male gender of rearing may be a viable option for parents whose children are born with congenital adrenal hyperplasia, a 46,XX karyotype and male genitalia, although positive parental and other support, and counseling are needed for adjustment. Copyright © 2010 American Urological Association Education and Research

[A novel homozygous mutation p.E25X in the HSD3B2 gene causing salt wasting 3β-hydroxysteroid dehydrogenases deficiency in a Chinese pubertal girl: a delayed diagnosis until recurrent ovary cysts].

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Huang, Yonglan; Zheng, Jipeng; Xie, Ting; Xiao, Qing; Lu, Shaomei; Li, Xiuzhen; Cheng, Jing; Chen, Lihe; Liu, Li

2014-12-01

3β- hydroxysteroid dehydrogenase deficiency (3βHSD), a rare form of congenital adrenal hyperplasia (CAH) resulted from mutations in the HSD3B2 gene that impair steroidogenesis in both adrenals and gonads. We report clinical features and the results of HSD3B2 gene analysis of a Chinese pubertal girl with salt wasting 3βHSD deficiency. We retrospectively reviewed clinical presentations and steroid profiles of the patient diagnosed in Guangzhou Women and Children's Medical Center in 2013. PCR and direct sequencing were used to identify any mutation in the HSD3B2 gene. A 13-year-old girl was diagnosed as CAH after birth because of salt-wasting with mild clitorimegaly and then was treated with glucocorticoid replacement. Breast and pubic hair development were normal, and menarche occurred at 12 yr, followed by menstrual bleeding about every 45 days. In the last one year laparoscopic operation and ovariocentesis were performed one after another for recurrent ovary cysts. Under corticoid acetate therapy, ACTH 17.10 pmol/L (normal 0-10.12), testosterone 1.31 nmol/L (normal T (p.E25X) was identified in HSD3B2 gene. The girl was homozygous and her mother was heterozygous, while her father was not identified with this mutation. A classic 3βHSD deficiency is characterized by salt wasting and mild virilization in female. Ovary cysts may be the one of features of gonad phenotype indicating ovary 3βHSD deficiency. A novel homozygous mutation c.73G >T(p.E25X) was related to the classical phenotype.

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

NARCIS (Netherlands)

Sanches, S.A.; Wiegers, T.A.; Otten, B.J.; Claahsen-van der Grinten, H.L.

2012-01-01

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

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

Energy Technology Data Exchange (ETDEWEB)

Stikkelbroeck, Nike M.M.L.; Schouten, Diana; Otten, Barto J. [University Medical Centre Nijmegen, Department of Paediatric Endocrinology, P.O. Box 9101, Nijmegen (Netherlands); Hermus, Ad R.M.M. [University Medical Centre Nijmegen, Department of Endocrinology, P.O. Box 9101, Nijmegen (Netherlands); Suliman, Harold M.; Jager, Gerrit J. [University Medical Centre Nijmegen, Department of Radiology, P.O. Box 9101, Nijmegen (Netherlands); Braat, Didi D.M. [University Medical Centre Nijmegen, Department of Obstetrics and Gynaecology, P.O. Box 9101, Nijmegen (Netherlands)

2004-10-01

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.)

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.)

Gender role across development in adult women with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

Science.gov (United States)

Long, Dominique N; Wisniewski, Amy B; Migeon, Claude J

2004-10-01

This study evaluated the degree of femininity and masculinity at different developmental stages in a group of adult women, some of whom were exposed to elevated prenatal adrenal androgens as a result of congenital adrenal hyperplasia (CAH) due to 21 hydroxylase (21-OH) deficiency. Women who had presented to the Johns Hopkins Hospital Pediatric Endocrine Clinic for treatment of CAH due to 21-OH deficiency were included. The control group consisted of sisters of CAH participants and women referred for evaluation of polycystic ovary syndrome. Study participants were given a questionnaire asking them to indicate their degree of masculinity and femininity during childhood, adolescence, and adulthood. In addition, participants were asked questions related to their play behavior during childhood, including playmate preferences, toy preferences, and admiration of male or female characters during fantasy play. Across participant groups, self-reported femininity decreased in a dose response manner, according to prenatal androgen exposure. For all groups, femininity increased through developmental stages. Women with salt-losing CAH remained less feminine than controls into adulthood. Conversely, self-reported masculinity increased in a dose-response manner, according to prenatal androgen exposure, across participant groups. Women with CAH showed a decrease in masculinity across developmental stages, such that by adulthood, there were no significant differences in masculinity between controls and the women with CAH. Women with salt-losing CAH were more likely to recall preferences for boy playmates, male-typical toys, and admiration for male characters during childhood than other study participants. Our data support the effect of both prenatal androgen exposure and socialization on gender role behavior in adult women with CAH due to 21-OH deficiency.

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

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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.

Adrenal rest tissue in gonads of patients with classical congenital adrenal hyperplasia: multicenter study of 45 French male patients.

Science.gov (United States)

Pierre, Peggy; Despert, François; Tranquart, François; Coutant, Régis; Tardy, Véronique; Kerlan, Véronique; Sonnet, Emmanuel; Baron, Sabine; Lorcy, Yannick; Emy, Philippe; Delavierre, Dominique; Monceaux, Françoise; Morel, Yves; Lecomte, Pierre

2012-12-01

Several cases of testicular adrenal rest tumours have been reported in men with congenital adrenal hyperplasia (CAH) due to the classical form of 21-hydroxylase deficiency but the prevalence has not been established. The aims of this report were to evaluate the frequency of testicular adrenal rest tissue in this population in a retrospective multicentre study involving eight endocrinology centres, and to determine whether treatment or genetic background had an impact on the occurrence of adrenal rest tissue. Testicular adrenal rest tissue (TART) was sought clinically and with ultrasound examination in forty-five males with CAH due to the classical form of 21-hydroxylase deficiency. When the diagnosis of testicular adrenal rest tumours was sought, good observance of treatment was judged on biological concentrations of 17-hydroxyprogesterone (17OHP), delta4-androstenedione, active renin and testosterone. The results of affected and non-affected subjects were compared. TART was detected in none of the 18 subjects aged 1 to 15years but was detected in 14 of the 27 subjects aged more than 15years. Five patients with an abnormal echography result had no clinical signs. Therapeutic control evaluated at diagnosis of TART seemed less effective when diagnosis was made in patients with adrenal rest tissue compared to TART-free subjects. Various genotypes were observed in patients with or without TART. Due to the high prevalence of TART in classical CAH and the delayed clinical diagnosis, testicular ultrasonography must be performed before puberty and thereafter regularly during adulthood even if the clinical examination is normal. Copyright © 2012 Elsevier Masson SAS. All rights reserved.

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.

New mutations of DAX-1 genes in two Japanese patients with X-linked congenital adrenal hypoplasia and hypogonadotropic hypogonadism

Energy Technology Data Exchange (ETDEWEB)

Yanase, Toshihiko; Takayanagi, Ryoichi; Oba, Koichi [Kyushu Univ., Fukuoka (Japan)] [and others

1996-02-01

Congenital adrenal hypoplasia, an X-linked disorder, is characterized by primary adrenal insufficiency and frequent association with hypogonadotropic hypogonadism. The X-chromosome gene DAX-1 has been most recently identified and shown to be responsible for this disorder. We analyzed the DAX-1 genes of two unrelated Japanese patients with congenital adrenal hypoplasia and hypogonadotropic hypogonadism by using PCR amplification of genomic DNA and its complete exonic sequencing. In a family containing several affected individuals, the proband male patient had a stop codon (TGA) in place of tryptophan (TGG) at amino acid position 171. As expected, his mother was a heterozygous carrier for the mutation, whereas his father and unaffected brother did not carry this mutation. In another male patient with noncontributory family history, sequencing revealed a 1-bp (T) deletion at amino acid position 280, leading to a frame shift and, subsequently a premature stop codon at amino acid position 371. The presence of this mutation in the patients` genome was further confirmed by digestion of genomic PCR product with MspI created by this mutation. Family studies using MspI digestion of genomic PCR products revealed that neither parent of this individual carried the mutation. These results clearly indicate that congenital adrenal hypoplasia and hypogonadotropic hypogonadism result from not only inherited but also de novo mutation in the DAX-1 gene. 31 refs., 4 figs., 2 tabs.

Influence of hormonal control on LH pulsatility and secretion in women with classical congenital adrenal hyperplasia.

Science.gov (United States)

Bachelot, Anne; Chakhtoura, Zeina; Plu-Bureau, Geneviève; Coudert, Mathieu; Coussieu, Christiane; Badachi, Yasmina; Dulon, Jérome; Charbit, Beny; Touraine, Philippe

2012-10-01

Women with classical congenital adrenal hyperplasia (CAH) exhibit reduced fertility due to several factors including anovulation. This has been attributed to a disturbed gonadotropic axis as in polycystic ovary syndrome (PCOS), but there is no precise evaluation. Our aim was to evaluate the gonadotropic axis and LH pulsatility patterns and to determine factor(s) that could account for the potential abnormality of LH pulsatility. Case/control study. Sixteen CAH women (11 with the salt-wasting form and five with the simple virilizing form), aged from 18 to 40 years, and 16 age-matched women, with regular menstrual cycles (28 ± 3 days), were included. LH pulse patterns over 6 h were determined in patients and controls. No differences were observed between patients and controls in terms of mean LH levels, LH pulse amplitude, or LH frequency. In CAH patients, LH pulsatility patterns were heterogeneous, leading us to perform a clustering analysis of LH data, resulting in a two-cluster partition. Patients in cluster 1 had similar LH pulsatility patterns to the controls. Patients in cluster 2 had: lower LH pulse amplitude and frequency and presented menstrual cycle disturbances more frequently; higher 17-OH progesterone, testosterone, progesterone, and androstenedione levels; and lower FSH levels. LH pulsatility may be normal in CAH women well controlled by hormonal treatment. Undertreatment is responsible for hypogonadotropic hypogonadism, with low LH pulse levels and frequency, but not PCOS. Suppression of progesterone and androgen concentrations during the follicular phase of the menstrual cycle should be a major objective in these patients.

Low-Dose Dexamethasone Therapy from Infancy of Virilizing Congenital Adrenal Hyperplasia

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Stephenson Kerry

2009-12-01

Full Text Available Objective. To assess the growth and control of adrenal androgen secretion in children with virilizing congenital adrenal hyperplasia (CAH treated with dexamethasone. Method. We examined doses used, control of adrenal androgen secretion, and growth and skeletal maturation of 8 children with CAH treated with dexamethasone beginning in infancy. Results. 3 boys and 5 girls with classical CAH (17-hydroxyprogesterone at diagnosis >20,000 ng/dL were treated with dexamethasone beginning at diagnosis ( ; all doses were given in the morning using a dosing syringe to administer a 0.1 mg/mL elixir. The children were treated for years over which time the change in bone age to chronological age ratio (BA/CA was . Most recent height Z' scores were , and body mass index (BMI scores were . Late afternoon levels of 17-hydroxyprogesterone, androstenedione, and testosterone were  ng/dL ( nmol/L,  ng/dL ( nmol/L, and  ng/dL; ( nmol/L, respectively. Conclusions. These observations show that low doses of dexamethasone can be used to effectively treat CAH beginning in infancy.

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.

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.

Molten salt oxidation of organic hazardous waste with high salt content.

Science.gov (United States)

Lin, Chengqian; Chi, Yong; Jin, Yuqi; Jiang, Xuguang; Buekens, Alfons; Zhang, Qi; Chen, Jian

2018-02-01

Organic hazardous waste often contains some salt, owing to the widespread use of alkali salts during industrial manufacturing processes. These salts cause complications during the treatment of this type of waste. Molten salt oxidation is a flameless, robust thermal process, with inherent capability of destroying the organic constituents of wastes, while retaining the inorganic ingredients in the molten salt. In the present study, molten salt oxidation is employed for treating a typical organic hazardous waste with a high content of alkali salts. The hazardous waste derives from the production of thiotriazinone. Molten salt oxidation experiments have been conducted using a lab-scale molten salt oxidation reactor, and the emissions of CO, NO, SO 2 , HCl and dioxins are studied. Impacts are investigated from the composition of the molten salts, the types of feeding tube, the temperature of molten carbonates and the air factor. Results show that the waste can be oxidised effectively in a molten salt bath. Temperature of molten carbonates plays the most important role. With the temperature rising from 600 °C to 750 °C, the oxidation efficiency increases from 91.1% to 98.3%. Compared with the temperature, air factor has but a minor effect, as well as the composition of the molten salts and the type of feeding tube. The molten carbonates retain chlorine with an efficiency higher than 99.9% and the emissions of dioxins are below 8 pg TEQ g -1 sample. The present study shows that molten salt oxidation is a promising alternative for the disposal of organic hazardous wastes containing a high salt content.

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

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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.

Designing Advanced Ceramic Waste Forms for Electrochemical Processing Salt Waste

International Nuclear Information System (INIS)

Ebert, W. L.; Snyder, C. T.; Frank, Steven; Riley, Brian

2016-01-01

This report describes the scientific basis underlying the approach being followed to design and develop ''advanced'' glass-bonded sodalite ceramic waste form (ACWF) materials that can (1) accommodate higher salt waste loadings than the waste form developed in the 1990s for EBR-II waste salt and (2) provide greater flexibility for immobilizing extreme waste salt compositions. This is accomplished by using a binder glass having a much higher Na_2O content than glass compositions used previously to provide enough Na+ to react with all of the Cl- in the waste salt and generate the maximum amount of sodalite. The phase compositions and degradation behaviors of prototype ACWF products that were made using five new binder glass formulations and with 11-14 mass% representative LiCl/KCl-based salt waste were evaluated and compared with results of similar tests run with CWF products made using the original binder glass with 8 mass% of the same salt to demonstrate the approach and select a composition for further studies. About twice the amount of sodalite was generated in all ACWF materials and the microstructures and degradation behaviors confirmed our understanding of the reactions occurring during waste form production and the efficacy of the approach. However, the porosities of the resulting ACWF materials were higher than is desired. These results indicate the capacity of these ACWF waste forms to accommodate LiCl/KCl-based salt wastes becomes limited by porosity due to the low glass-to-sodalite volume ratio. Three of the new binder glass compositions were acceptable and there is no benefit to further increasing the Na content as initially planned. Instead, further studies are needed to develop and evaluate alternative production methods to decrease the porosity, such as by increasing the amount of binder glass in the formulation or by processing waste forms in a hot isostatic press. Increasing the amount of binder glass to eliminate porosity will decrease the waste

Designing Advanced Ceramic Waste Forms for Electrochemical Processing Salt Waste

Energy Technology Data Exchange (ETDEWEB)

Ebert, W. L. [Argonne National Lab. (ANL), Argonne, IL (United States); Snyder, C. T. [Argonne National Lab. (ANL), Argonne, IL (United States); Frank, Steven [Argonne National Lab. (ANL), Argonne, IL (United States); Riley, Brian [Argonne National Lab. (ANL), Argonne, IL (United States)

2016-03-01

This report describes the scientific basis underlying the approach being followed to design and develop “advanced” glass-bonded sodalite ceramic waste form (ACWF) materials that can (1) accommodate higher salt waste loadings than the waste form developed in the 1990s for EBR-II waste salt and (2) provide greater flexibility for immobilizing extreme waste salt compositions. This is accomplished by using a binder glass having a much higher Na₂O content than glass compositions used previously to provide enough Na+ to react with all of the Cl– in the waste salt and generate the maximum amount of sodalite. The phase compositions and degradation behaviors of prototype ACWF products that were made using five new binder glass formulations and with 11-14 mass% representative LiCl/KCl-based salt waste were evaluated and compared with results of similar tests run with CWF products made using the original binder glass with 8 mass% of the same salt to demonstrate the approach and select a composition for further studies. About twice the amount of sodalite was generated in all ACWF materials and the microstructures and degradation behaviors confirmed our understanding of the reactions occurring during waste form production and the efficacy of the approach. However, the porosities of the resulting ACWF materials were higher than is desired. These results indicate the capacity of these ACWF waste forms to accommodate LiCl/KCl-based salt wastes becomes limited by porosity due to the low glass-to-sodalite volume ratio. Three of the new binder glass compositions were acceptable and there is no benefit to further increasing the Na content as initially planned. Instead, further studies are needed to develop and evaluate alternative production methods to decrease the porosity, such as by increasing the amount of binder glass in the formulation or by processing waste forms in a hot isostatic press. Increasing the amount of binder glass to eliminate porosity will decrease

Nonclassical Congenital Adrenal Hyperplasia and Pregnancy

Directory of Open Access Journals (Sweden)

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.

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)

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

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.

Waste treatment using molten salt oxidation

International Nuclear Information System (INIS)

Navratil, J.D.; Stewart, A.E.

1996-01-01

MSO technology can be characterized as a submerged oxidation process; the basic concept is to introduce air and wastes into a bed of molten salt, oxidize the organic wastes in the molten salt, use the heat of oxidation to keep the salt molten and remove the salt for disposal or processing and recycling. The molten salt (usually sodium carbonate at 900-1000 C) provides four waste management functions: providing a heat transfer medium, catalyzing the oxidation reaction, preventing the formation of acid gases by forming stable salts, and efficiently capturing ash particles and radioactive materials by the combined effects of wetting, encapsulation and dissolution. The MSO process requires no wet scrubbing system for off-gas treatment. The process has been developed through bench-scale and pilot-scale testing, with successful destruction demonstration of a wide variety of hazardous and mixed (radioactive and hazardous wastes). (author). 24 refs, 2 tabs, 2 figs

Zonal corticosteroid hormone biosynthesis in the adrenal cortex in rats exposed to emotional stress combined with salt loading

International Nuclear Information System (INIS)

Shul'ga, V.A.

1987-01-01

The authors study the pattern of biosynthesis of corticosteroid hormones in the zona glomerulosa and the combined zona fasciculata + zona reticularis of the adrenals, which are responsible for the mineralocorticoid and glucocorticoid function of the glands, during simultaneous exposure of animals to salt loading and emotional stress. Experiments were carried out on rats. The adrenals were divided into parts and samples were incubated in vitro with the addition of 3 H-progesterone to each sample. The specific activity of the 3 H-labeled corticosteroids decreased significantly in rats with a normal salt intake exposed to emotional stress

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

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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.

Hiperplasia adrenal congénita clásica: Tratamiento médico y quirúrgico Congenital clasic adrenal hyperplasia: Medical and surgical treatment

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José Raúl Zaldívar Ochoa

Full Text Available Se presenta el caso clínico de una infante de 2 años de edad que nació con genitales ambiguos y fue diagnosticada precozmente en el Hospital Infantil Norte Docente "Juan de la Cruz Martínez Maceira", gracias al Programa de Hiperplasia Adrenal Congénita. Se le realizó una exitosa reconstrucción quirúrgica hacia el sexo femenino y se brindó apoyo psicológico a la familia.The case report of a 2 year-old infant girl is presented who was born with ambiguous genitals and she was early diagnosed at "Juan de la Cruz Martínez Maceira" Teaching Nothern Pediatric Hospital, thanks to the Congenital Adrenal Hyperplasia Program. She had a successful surgical reconstruction to the female sex and her family received psychological support.

Management issues of congenital adrenal hyperplasia during the transition from pediatric to adult care.

Science.gov (United States)

Choi, Jin-Ho; Yoo, Han-Wook

2017-02-01

Steroid 21-hydroxylase deficiency is the most prevalent form of congenital adrenal hyperplasia (CAH), accounting for approximately 95% of cases. With the advent of newborn screening and hormone replacement therapy, most children with CAH survive into adulthood. Adolescents and adults with CAH experience a number of complications, including short stature, obesity, infertility, tumor, osteoporosis, and reduced quality of life. Transition from pediatric to adult care and management of long-term complications are challenging for both patients and health-care providers. Psychosocial issues frequently affect adherence to glucocorticoid treatment. Therefore, the safe transition of adolescents to adult care requires regular follow-up of patients by a multidisciplinary team including pediatric and adult endocrinologists. The major goals for management of adults with 21-hydroxylase deficiency are to minimize the long-term complications of glucocorticoid therapy, reduce hyperandrogenism, prevent adrenal or testicular adrenal rest tumors, maintain fertility, and improve quality of life. Optimized medical or surgical treatment strategies should be developed through coordinated care, both during transition periods and throughout patients' lifetimes. This review will summarize current knowledge on the management of adults with CAH, and suggested appropriate approaches to the transition from pediatric to adult care.

Functional studies of novel CYP21A2 mutations detected in Norwegian patients with congenital adrenal hyperplasia

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Brønstad, Ingeborg; Breivik, Lars; Methlie, Paal; Wolff, Anette S B; Bratland, Eirik; Nermoen, Ingrid; Løvås, Kristian; Husebye, Eystein S

2014-01-01

In about 95% of cases, congenital adrenal hyperplasia (CAH) is caused by mutations in CYP21A2 gene encoding steroid 21-hydroxylase (21OH). Recently, we have reported four novel CYP21A2 variants in the Norwegian population of patients with CAH, of which p.L388R and p.E140K were associated with salt wasting (SW), p.P45L with simple virilising (SV) and p.V211M+p.V281L with SV to non-classical (NC) phenotypes. We aimed to characterise the novel variants functionally utilising a newly designed in vitro assay of 21OH enzyme activity and structural simulations and compare the results with clinical phenotypes. CYP21A2 mutations and variants were expressed in vitro. Enzyme activity was assayed by assessing the conversion of 17-hydroxyprogesterone to 11-deoxycortisol by liquid chromatography tandem mass spectroscopy. PyMOL 1.3 was used for structural simulations, and PolyPhen2 and PROVEAN for predicting the severity of the mutants. The CYP21A2 mutants, p.L388R and p.E140K, exhibited 1.1 and 11.3% of wt 21OH enzyme activity, respectively, in vitro. We could not detect any functional deficiency of the p.P45L variant in vitro; although prediction tools suggest p.P45L to be pathogenic. p.V211M displayed enzyme activity equivalent to the wt in vitro, which was supported by in silico analyses. We found good correlations between phenotype and the in vitro enzyme activities of the SW mutants, but not for the SV p.P45L variant. p.V211M might have a synergistic effect together with p.V281L, explaining a phenotype between SV and NC CAH. PMID:24671123

Low-Dose Dexamethasone Therapy from Infancy of Virilizing Congenital Adrenal Hyperplasia

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Scott A. Rivkees

2009-01-01

Full Text Available Objective. To assess the growth and control of adrenal androgen secretion in children with virilizing congenital adrenal hyperplasia (CAH treated with dexamethasone. Method. We examined doses used, control of adrenal androgen secretion, and growth and skeletal maturation of 8 children with CAH treated with dexamethasone beginning in infancy. Results. 3 boys and 5 girls with classical CAH (17-hydroxyprogesterone at diagnosis >20,000 ng/dL were treated with dexamethasone beginning at diagnosis (<10 days of age. Patients were also treated with fludrocortisone and sodium chloride. The average initial medication dose was 0.1 mg (0.28±0.015 mg/m2; all doses were given in the morning using a dosing syringe to administer a 0.1 mg/mL elixir. The children were treated for 6.5±2.0 years over which time the change in bone age to chronological age ratio (ΔBA/ΔCA was 0.9±0.06. Most recent height Z' scores were +0.5±0.2, and body mass index (BMI scores were 18±0.2. Late afternoon levels of 17-hydroxyprogesterone, androstenedione, and testosterone were 780±238 ng/dL (23.4±7 nmol/L, 42±10 ng/dL (1.4±0.3 nmol/L, and 11.5±3 ng/dL; (0.4±0.1 nmol/L, respectively. Conclusions. These observations show that low doses of dexamethasone can be used to effectively treat CAH beginning in infancy.

Noninvasive Prenatal Diagnosis of Congenital Adrenal Hyperplasia.

Science.gov (United States)

Khattab, Ahmed; Yuen, Tony; Sun, Li; Yau, Mabel; Barhan, Ariella; Zaidi, Mone; Lo, Y M Dennis; New, Maria I

2016-01-01

A major hallmark of classical congenital adrenal hyperplasia (CAH) is genital ambiguity noted at birth in affected females, which leads to psychological and psychosexual issues in adult life. Attempts to correct genital ambiguity through surgical intervention have been partially successful. Fetal hyperandrogenemia and genital ambiguity have been shown to be preventable by prenatal administration of low-dose dexamethasone initiated before the 9th week of gestation. In 7 of 8 at-risk pregnancies, the unaffected fetus is unnecessarily exposed to dexamethasone for weeks until the diagnosis of classical CAH is ruled out by invasive procedures. This therapeutic dilemma calls for early prenatal diagnosis so that dexamethasone treatment can be directed to affected female fetuses only. We describe the utilization of cell-free fetal DNA in mothers carrying at-risk fetuses as early as 6 gestational weeks by targeted massively parallel sequencing of the genomic region including and flanking the CYP21A2 gene. Our highly personalized and innovative approach should permit the diagnosis of CAH before genital development begins, therefore restricting the purposeful administration of dexamethasone to mothers carrying affected females. © 2016 S. Karger AG, Basel.

Confirmation of congenital adrenal hyperplasia by adrenal steroid profiling of filter paper dried blood samples using ultra-performance liquid chromatography-tandem mass spectrometry.

Science.gov (United States)

Rossi, Claudia; Calton, Lisa; Brown, Heather A; Gillingwater, Scott; Wallace, A Michael; Petrucci, Francesca; Ciavardelli, Domenico; Urbani, Andrea; Sacchetta, Paolo; Morris, Michael

2011-04-01

The specificity of screening for congenital adrenal hyperplasia by direct measurement of 17-hydroxyprogesterone in filter paper dried blood spot samples by immunoassay is low and has a high false-positive rate. In order to reduce the false-positive rate of this test, we developed a rapid, robust, specific confirmatory procedure in which cortisol, 4-androstene-3,17-dione and 17-hydroxyprogesterone were measured simultaneously by ultra-performance liquid chromatography-tandem mass spectrometry. After extraction, samples were analysed by ultra-performance liquid chromatography-tandem mass spectrometry and 17-hydroxyprogesterone was quantified accurately. Other steroids were determined using stable deuterated internal standards. In total, 25 patient blood spot samples and 92 control samples were analysed. The assay was linear for 17-hydroxyprogesterone, with a coefficient of determination >0.997 and imprecision ≤ 6.5%. An upper limit of normal for 17-hydroxyprogester-one of 4.45 nmol/L was established by analysing a cohort of samples from unaffected newborns. In addition, a cut-off of 3.5 for the peak areas ratio (17-hydroxyprogesterone+4-androstene-3,17-dione)/cortisol, allows confirmation of the affected steroidogenic enzyme. A high throughput method for the detection of steroids related to congenital adrenal hyperplasia has been developed, allowing the false-positive rate associated with screening for 17-hydroxyprogesterone by immunoassay to be determined.

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.

Salt disposal of heat-generating nuclear waste

International Nuclear Information System (INIS)

Leigh, Christi D.; Hansen, Francis D.

2011-01-01

This report summarizes the state of salt repository science, reviews many of the technical issues pertaining to disposal of heat-generating nuclear waste in salt, and proposes several avenues for future science-based activities to further the technical basis for disposal in salt. There are extensive salt formations in the forty-eight contiguous states, and many of them may be worthy of consideration for nuclear waste disposal. The United States has extensive experience in salt repository sciences, including an operating facility for disposal of transuranic wastes. The scientific background for salt disposal including laboratory and field tests at ambient and elevated temperature, principles of salt behavior, potential for fracture damage and its mitigation, seal systems, chemical conditions, advanced modeling capabilities and near-future developments, performance assessment processes, and international collaboration are all discussed. The discussion of salt disposal issues is brought current, including a summary of recent international workshops dedicated to high-level waste disposal in salt. Lessons learned from Sandia National Laboratories' experience on the Waste Isolation Pilot Plant and the Yucca Mountain Project as well as related salt experience with the Strategic Petroleum Reserve are applied in this assessment. Disposal of heat-generating nuclear waste in a suitable salt formation is attractive because the material is essentially impermeable, self-sealing, and thermally conductive. Conditions are chemically beneficial, and a significant experience base exists in understanding this environment. Within the period of institutional control, overburden pressure will seal fractures and provide a repository setting that limits radionuclide movement. A salt repository could potentially achieve total containment, with no releases to the environment in undisturbed scenarios for as long as the region is geologically stable. Much of the experience gained from United

Salt disposal of heat-generating nuclear waste.

Energy Technology Data Exchange (ETDEWEB)

Leigh, Christi D. (Sandia National Laboratories, Carlsbad, NM); Hansen, Francis D.

2011-01-01

This report summarizes the state of salt repository science, reviews many of the technical issues pertaining to disposal of heat-generating nuclear waste in salt, and proposes several avenues for future science-based activities to further the technical basis for disposal in salt. There are extensive salt formations in the forty-eight contiguous states, and many of them may be worthy of consideration for nuclear waste disposal. The United States has extensive experience in salt repository sciences, including an operating facility for disposal of transuranic wastes. The scientific background for salt disposal including laboratory and field tests at ambient and elevated temperature, principles of salt behavior, potential for fracture damage and its mitigation, seal systems, chemical conditions, advanced modeling capabilities and near-future developments, performance assessment processes, and international collaboration are all discussed. The discussion of salt disposal issues is brought current, including a summary of recent international workshops dedicated to high-level waste disposal in salt. Lessons learned from Sandia National Laboratories' experience on the Waste Isolation Pilot Plant and the Yucca Mountain Project as well as related salt experience with the Strategic Petroleum Reserve are applied in this assessment. Disposal of heat-generating nuclear waste in a suitable salt formation is attractive because the material is essentially impermeable, self-sealing, and thermally conductive. Conditions are chemically beneficial, and a significant experience base exists in understanding this environment. Within the period of institutional control, overburden pressure will seal fractures and provide a repository setting that limits radionuclide movement. A salt repository could potentially achieve total containment, with no releases to the environment in undisturbed scenarios for as long as the region is geologically stable. Much of the experience gained from

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

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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.

Mixed Waste Salt Encapsulation Using Polysiloxane - Final Report

International Nuclear Information System (INIS)

Miller, C.M.; Loomis, G.G.; Prewett, S.W.

1997-01-01

A proof-of-concept experimental study was performed to investigate the use of Orbit Technologies polysiloxane grouting material for encapsulation of U.S. Department of Energy mixed waste salts leading to a final waste form for disposal. Evaporator pond salt residues and other salt-like material contaminated with both radioactive isotopes and hazardous components are ubiquitous in the DOE complex and may exceed 250,000,000 kg of material. Current treatment involves mixing low waste percentages (less than 10% by mass salt) with cement or costly thermal treatment followed by cementation to the ash residue. The proposed technology involves simple mixing of the granular salt material (with relatively high waste loadings-greater than 50%) in a polysiloxane-based system that polymerizes to form a silicon-based polymer material. This study involved a mixing study to determine optimum waste loadings and compressive strengths of the resultant monoliths. Following the mixing study, durability testing was performed on promising waste forms. Leaching studies including the accelerated leach test and the toxicity characteristic leaching procedure were also performed on a high nitrate salt waste form. In addition to this testing, the waste form was examined by scanning electron microscope. Preliminary cost estimates for applying this technology to the DOE complex mixed waste salt problem is also given

Projected Salt Waste Production from a Commercial Pyroprocessing Facility

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Michael F. Simpson

2013-01-01

Full Text Available Pyroprocessing of used nuclear fuel inevitably produces salt waste from electrorefining and/or oxide reduction unit operations. Various process design characteristics can affect the actual mass of such waste produced. This paper examines both oxide and metal fuel treatment, estimates the amount of salt waste generated, and assesses potential benefit of process options to mitigate the generation of salt waste. For reference purposes, a facility is considered in which 100 MT/year of fuel is processed. Salt waste estimates range from 8 to 20 MT/year from considering numerous scenarios. It appears that some benefit may be derived from advanced processes for separating fission products from molten salt waste, but the degree of improvement is limited. Waste form production is also considered but appears to be economically unfavorable. Direct disposal of salt into a salt basin type repository is found to be the most promising with respect to minimizing the impact of waste generation on the economic feasibility and sustainability of pyroprocessing.

Cementitious Stabilization of Mixed Wastes with High Salt Loadings

International Nuclear Information System (INIS)

Spence, R.D.; Burgess, M.W.; Fedorov, V.V.; Downing, D.J.

1999-01-01

Salt loadings approaching 50 wt % were tolerated in cementitious waste forms that still met leach and strength criteria, addressing a Technology Deficiency of low salt loadings previously identified by the Mixed Waste Focus Area. A statistical design quantified the effect of different stabilizing ingredients and salt loading on performance at lower loadings, allowing selection of the more effective ingredients for studying the higher salt loadings. In general, the final waste form needed to consist of 25 wt % of the dry stabilizing ingredients to meet the criteria used and 25 wt % water to form a workable paste, leaving 50 wt % for waste solids. The salt loading depends on the salt content of the waste solids but could be as high as 50 wt % if all the waste solids are salt

Comparison of Clinico-Radiological Features between Congenital Cystic Neuroblastoma and Neonatal Adrenal Hemorrhagic Pseudocyst

Energy Technology Data Exchange (ETDEWEB)

Eo, Hong; Kim, Ji Hye; Jang, Kyung Mi; Yoo, So Young [Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of); Lim, Gye Yeon [St. Mary' s Hospital Catholic University, Seoul (Korea, Republic of); Kim, Myung Joon [Severance Hospital Yonsei University, Seoul (Korea, Republic of); Kim, Ok Hwa [Ajou University Hospital, Suwon (Korea, Republic of)

2011-02-15

To evaluate the radiological and clinical findings of congenital cystic neuroblastomas as compared with those of the cystic presentation of neonatal adrenal hemorrhage. We analyzed the US (n = 52), CT (n = 24), and MR (n = 4) images as well as the medical records of 28 patients harboring congenital cystic neuroblastomas (n = 16) and neonatal adrenal hemorrhagic pseudocysts (n = 14). The history of prenatal detection, location, size, presence of outer wall enhancement, internal septations, solid portion, calcification, turbidity, vascular flow on a Doppler examination, and evolution patterns were compared in two groups of cystic lesions, by Fischer's exact test. All (100%) neuroblastomas and three (21%) of the 14 hemorrhagic pseudocysts were detected prenatally. Both groups of cystic lesions occurred more frequently on the right side; 11 of 16 (69%) for neuroblastomas and 11 of 14 (79%) for hemorrhagic pseudocysts. The size, presence of solid portion, septum, enhancement, and turbidity did not differ significantly (p > 0.05) between the two groups of cystic lesions. However, tiny calcifications (n = 3) and vascular flow on color Doppler US (n = 3) were noted in only neuroblastomas. The cystic neuroblastomas became complex solid and cystic masses, and did not disappear for up to 90 days in the three following cases, whereas 11 of the 14 (79%) hemorrhagic pseudocysts disappeared completely and the three remaining (27%) evolved to calcifications only. Although the imaging findings of two groups of cystic lesions were similar, prenatal detection, the presence of calcification on initial images, vascularity on color Doppler US, and evolution to a more complex mass may all favor neuroblastomas

Comparison of Clinico-Radiological Features between Congenital Cystic Neuroblastoma and Neonatal Adrenal Hemorrhagic Pseudocyst

International Nuclear Information System (INIS)

Eo, Hong; Kim, Ji Hye; Jang, Kyung Mi; Yoo, So Young; Lim, Gye Yeon; Kim, Myung Joon; Kim, Ok Hwa

2011-01-01

To evaluate the radiological and clinical findings of congenital cystic neuroblastomas as compared with those of the cystic presentation of neonatal adrenal hemorrhage. We analyzed the US (n = 52), CT (n = 24), and MR (n = 4) images as well as the medical records of 28 patients harboring congenital cystic neuroblastomas (n = 16) and neonatal adrenal hemorrhagic pseudocysts (n = 14). The history of prenatal detection, location, size, presence of outer wall enhancement, internal septations, solid portion, calcification, turbidity, vascular flow on a Doppler examination, and evolution patterns were compared in two groups of cystic lesions, by Fischer's exact test. All (100%) neuroblastomas and three (21%) of the 14 hemorrhagic pseudocysts were detected prenatally. Both groups of cystic lesions occurred more frequently on the right side; 11 of 16 (69%) for neuroblastomas and 11 of 14 (79%) for hemorrhagic pseudocysts. The size, presence of solid portion, septum, enhancement, and turbidity did not differ significantly (p > 0.05) between the two groups of cystic lesions. However, tiny calcifications (n = 3) and vascular flow on color Doppler US (n = 3) were noted in only neuroblastomas. The cystic neuroblastomas became complex solid and cystic masses, and did not disappear for up to 90 days in the three following cases, whereas 11 of the 14 (79%) hemorrhagic pseudocysts disappeared completely and the three remaining (27%) evolved to calcifications only. Although the imaging findings of two groups of cystic lesions were similar, prenatal detection, the presence of calcification on initial images, vascularity on color Doppler US, and evolution to a more complex mass may all favor neuroblastomas

Alternatives for definse waste-salt disposal

International Nuclear Information System (INIS)

Benjamin, R.W.; McDonell, W.R.

1983-01-01

Alternatives for disposal of decontaminated high-level waste salt at Savannah River were reviewed to estimate costs and potential environmental impact for several processes. In this review, the reference process utilizing intermediate-depth burial of salt-concrete (saltcrete) monoliths was compared with alternatives including land application of the decontaminated salt as fertilizer for SRP pine stands, ocean disposal with and without containment, and terminal storage as saltcake in existing SRP waste tanks. Discounted total costs for the reference process and its modifications were in the same range as those for most of the alternative processes; uncontained ocean disposal with truck transport to Savannah River barges and storage as saltcake in SRP tanks had lower costs, but presented other difficulties. Environmental impacts could generally be maintained within acceptable limits for all processes except retention of saltcake in waste tanks, which could result in chemical contamination of surrounding areas on tank collapse. Land application would require additional salt decontamination to meet radioactive waste disposal standards, and ocean disposal without containment is not permitted in existing US practice. The reference process was judged to be the only salt disposal option studied which would meet all current requirements at an acceptable cost

Organic waste processing using molten salt oxidation

Energy Technology Data Exchange (ETDEWEB)

Adamson, M. G., LLNL

1998-03-01

Molten Salt Oxidation (MSO) is a thermal means of oxidizing (destroying) the organic constituents of mixed wastes, hazardous wastes, and energetic materials while retaining inorganic and radioactive constituents in the salt. For this reason, MSO is considered a promising alternative to incineration for the treatment of a variety of organic wastes. The U. S. Department of Energy`s Office of Environmental Management (DOE/EM) is currently funding research that will identify alternatives to incineration for the treatment of organic-based mixed wastes. (Mixed wastes are defined as waste streams which have both hazardous and radioactive properties.) One such project is Lawrence Livermore National Laboratory`s Expedited Technology Demonstration of Molten Salt Oxidation (MSO). The goal of this project is to conduct an integrated demonstration of MSO, including off-gas and spent salt treatment, and the preparation of robust solid final forms. Livermore National Laboratory (LLNL) has constructed an integrated pilot-scale MSO treatment system in which tests and demonstrations are presently being performed under carefully controlled (experimental) conditions. The system consists of a MSO process vessel with dedicated off-gas treatment, a salt recycle system, feed preparation equipment, and equipment for preparing ceramic final waste forms. In this paper we describe the integrated system and discuss its capabilities as well as preliminary process demonstration data. A primary purpose of these demonstrations is to identify the most suitable waste streams and waste types for MSO treatment.

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

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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.

Prenatal Hormones and Postnatal Socialization by Parents as Determinants of Male-Typical Toy Play in Girls With Congenital Adrenal Hyperplasia

Science.gov (United States)

Pasterski, Vickie L.; Geffner, Mitchell E.; Brain, Caroline; Hindmarsh, Peter; Brook, Charles; Hines, Melissa

2005-01-01

Toy choices of 3- to 10-year-old children with congenital adrenal hyperplasia (CAH) and of their unaffected siblings were assessed. Also assessed was parental encouragement of sex-typed toy play. Girls with CAH displayed more male-typical toy choices than did their unaffected sisters, whereas boys with and without CAH did not differ. Mothers and…

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.

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

Directory of Open Access Journals (Sweden)

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.

Disposal of Savannah River Plant waste salt

International Nuclear Information System (INIS)

Dukes, M.D.

1982-01-01

Approximately 26-million gallons of soluble low-level waste salts will be produced during solidification of 6-million gallons of high-level defense waste in the proposed Defense Waste Processing Facility (DWPF) at the Savannah River Plant (SRP). Soluble wastes (primarily NaNO 3 , NaNO 2 , and NaOH) stored in the waste tanks will be decontaminated by ion exchange and solidified in concrete. The resulting salt-concrete mixture, saltcrete, will be placed in a landfill on the plantsite such that all applicable federal and state disposal criteria are met. Proposed NRC guidelines for the disposal of waste with the radionuclide content of SRP salt would permit shallow land burial. Federal and state rules require that potentially hazardous chemical wastes (mainly nitrate-nitrate salts in the saltcrete) be contained to the degree necessary to meet drinking water standards in the ground water beneath the landfill boundary. This paper describes the proposed saltcrete landfill and tests under way to ensure that the landfill will meet these criteria. The work includes laboratory and field tests of the saltcrete itself, a field test of a one-tenth linear scale model of the entire landfill system, and a numerical model of the system

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

Directory of Open Access Journals (Sweden)

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.

Solid waste disposal into salt mines

International Nuclear Information System (INIS)

Repke, W.

1981-01-01

The subject is discussed as follows: general introduction to disposal of radioactive waste; handling of solid nuclear waste; technology of final disposal, with specific reference to salt domes; conditioning of radioactive waste; safety barriers for radioactive waste; practice of final disposal in other countries. (U.K.)

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.

Integrated demonstration of molten salt oxidation with salt recycle for mixed waste treatment

International Nuclear Information System (INIS)

Hsu, P.C.

1997-01-01

Molten Salt Oxidation (MSO) is a thermal, nonflame process that has the inherent capability of completely destroying organic constituents of mixed wastes, hazardous wastes, and energetic materials while retaining inorganic and radioactive constituents in the salt. For this reason, MSO is considered a promising alternative to incineration for the treatment of a variety of organic wastes. Lawrence Livermore National Laboratory (LLNL) has prepared a facility and constructed an integrated pilot-scale MSO treatment system in which tests and demonstrations are performed under carefully controlled (experimental) conditions. The system consists of a MSO processor with dedicated off-gas treatment, a salt recycle system, feed preparation equipment, and equipment for preparing ceramic final waste forms. This integrated system was designed and engineered based on laboratory experience with a smaller engineering-scale reactor unit and extensive laboratory development on salt recycle and final forms preparation. In this paper we present design and engineering details of the system and discuss its capabilities as well as preliminary process demonstration data. A primary purpose of these demonstrations is identification of the most suitable waste streams and waste types for MSO treatment

Permanent Disposal of Nuclear Waste in Salt

Science.gov (United States)

Hansen, F. D.

2016-12-01

Salt formations hold promise for eternal removal of nuclear waste from our biosphere. Germany and the United States have ample salt formations for this purpose, ranging from flat-bedded formations to geologically mature dome structures. Both nations are revisiting nuclear waste disposal options, accompanied by extensive collaboration on applied salt repository research, design, and operation. Salt formations provide isolation while geotechnical barriers reestablish impermeability after waste is placed in the geology. Between excavation and closure, physical, mechanical, thermal, chemical, and hydrological processes ensue. Salt response over a range of stress and temperature has been characterized for decades. Research practices employ refined test techniques and controls, which improve parameter assessment for features of the constitutive models. Extraordinary computational capabilities require exacting understanding of laboratory measurements and objective interpretation of modeling results. A repository for heat-generative nuclear waste provides an engineering challenge beyond common experience. Long-term evolution of the underground setting is precluded from direct observation or measurement. Therefore, analogues and modeling predictions are necessary to establish enduring safety functions. A strong case for granular salt reconsolidation and a focused research agenda support salt repository concepts that include safety-by-design. Sandia National Laboratories is a multi-program laboratory managed and operated by Sandia Corporation, a wholly owned subsidiary of Lockheed Martin Corporation, for the U.S. Department of Energy's National Nuclear Security Administration under contract DE-AC04-94AL85000. Author: F. D. Hansen, Sandia National Laboratories

Roadmap for disposal of Electrorefiner Salt as Transuranic Waste.

Energy Technology Data Exchange (ETDEWEB)

Rechard, Robert P. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Trone, Janis R. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Kalinina, Elena Arkadievna [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Wang, Yifeng [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Hadgu, Teklu [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Sanchez, Lawrence C. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States)

2017-12-01

The experimental breeder reactor (EBR-II) used fuel with a layer of sodium surrounding the uranium-zirconium fuel to improve heat transfer. Disposing of EBR-II fuel in a geologic repository without treatment is not prudent because of the potentially energetic reaction of the sodium with water. In 2000, the US Department of Energy (DOE) decided to treat the sodium-bonded fuel with an electrorefiner (ER), which produces metallic uranium product, a metallic waste, mostly from the cladding, and the salt waste in the ER, which contains most of the actinides and fission products. Two waste forms were proposed for disposal in a mined repository; the metallic waste, which was to be cast into ingots, and the ER salt waste, which was to be further treated to produce a ceramic waste form. However, alternative disposal pathways for metallic and salt waste streams may reduce the complexity. For example, performance assessments show that geologic repositories can easily accommodate the ER salt waste without treating it to form a ceramic waste form. Because EBR-II was used for atomic energy defense activities, the treated waste likely meets the definition of transuranic waste. Hence, disposal at the Waste Isolation Pilot Plant (WIPP) in southern New Mexico, may be feasible. This report reviews the direct disposal pathway for ER salt waste and describes eleven tasks necessary for implementing disposal at WIPP, provided space is available, DOE decides to use this alternative disposal pathway in an updated environmental impact statement, and the State of New Mexico grants permission.

Molten salt treatment to minimize and optimize waste

International Nuclear Information System (INIS)

Gat, U.; Crosley, S.M.; Gay, R.L.

1993-01-01

A combination molten salt oxidizer (MSO) and molten salt reactor (MSR) is described for treatment of waste. The MSO is proposed for contained oxidization of organic hazardous waste, for reduction of mass and volume of dilute waste by evaporation of the water. The NTSO residue is to be treated to optimize the waste in terms of its composition, chemical form, mixture, concentration, encapsulation, shape, size, and configuration. Accumulations and storage are minimized, shipments are sized for low risk. Actinides, fissile material, and long-lived isotopes are separated and completely burned or transmuted in an MSR. The MSR requires no fuel element fabrication, accepts the materials as salts in arbitrarily small quantities enhancing safety, security, and overall acceptability

Mixing Modeling Analysis For SRS Salt Waste Disposition

International Nuclear Information System (INIS)

Lee, S.

2011-01-01

Nuclear waste at Savannah River Site (SRS) waste tanks consists of three different types of waste forms. They are the lighter salt solutions referred to as supernate, the precipitated salts as salt cake, and heavier fine solids as sludge. The sludge is settled on the tank floor. About half of the residual waste radioactivity is contained in the sludge, which is only about 8 percentage of the total waste volume. Mixing study to be evaluated here for the Salt Disposition Integration (SDI) project focuses on supernate preparations in waste tanks prior to transfer to the Salt Waste Processing Facility (SWPF) feed tank. The methods to mix and blend the contents of the SRS blend tanks were evalutaed to ensure that the contents are properly blended before they are transferred from the blend tank such as Tank 50H to the SWPF feed tank. The work consists of two principal objectives to investigate two different pumps. One objective is to identify a suitable pumping arrangement that will adequately blend/mix two miscible liquids to obtain a uniform composition in the tank with a minimum level of sludge solid particulate in suspension. The other is to estimate the elevation in the tank at which the transfer pump inlet should be located where the solid concentration of the entrained fluid remains below the acceptance criterion (0.09 wt% or 1200 mg/liter) during transfer operation to the SWPF. Tank 50H is a Waste Tank that will be used to prepare batches of salt feed for SWPF. The salt feed must be a homogeneous solution satisfying the acceptance criterion of the solids entrainment during transfer operation. The work described here consists of two modeling areas. They are the mixing modeling analysis during miscible liquid blending operation, and the flow pattern analysis during transfer operation of the blended liquid. The modeling results will provide quantitative design and operation information during the mixing/blending process and the transfer operation of the blended

Waste salt recovery, recycle, and destruction

International Nuclear Information System (INIS)

Hickman, R.G.

1992-12-01

Starting in 1943 and continuing into the 1970s, radioactive wastes resulting from plutonium processing at Hanford were stored underground in 149 single shell tanks. Of these tanks, 66 are known or believedto be leaking, and over a period are believed to have leaked about 750,000 gal into the surrounding soil. The bulk of the aqueous solution has been removed and transferred to double shell tanks, none of which are leaking. The waste consists of 37 million gallons of salt cake and sludge. Most of the salt cake is sodium nitrate and other sodium salts. A substantial fraction of the sludge is sodium nitrate. Small amounts of the radionuclides are present in the sludge as oxides or hydroxides. In addition, some of the tanks contain organic compounds and ferrocyanide complexes, many of which have undergone radiolytic induced chemical changes during the years of storage. As part of the Hanford site remediation effort, the tank wastes must be removed, treated, and the residuals must be immobilized and disposed of in an environmentally acceptable manner. Removal methods of the waste from the tanks fall generally into three approaches: dry removal, slurry removal, and solution removed. The latter two methods are likely to result in some additional leakage to the surrounding soil, but that may be acceptable if the tank can be emptied and remediated before the leaked material permeates deeply into the soil. This effort includes three parts: salt splitting, acid separation, and destruction, with initial emphasis on salt splitting

Molten salt hazardous waste disposal process utilizing gas/liquid contact for salt recovery

International Nuclear Information System (INIS)

Grantham, L.F.; McKenzie, D.E.

1984-01-01

The products of a molten salt combustion of hazardous wastes are converted into a cooled gas, which can be filtered to remove hazardous particulate material, and a dry flowable mixture of salts, which can be recycled for use in the molten salt combustion, by means of gas/liquid contact between the gaseous products of combustion of the hazardous waste and a solution produced by quenching the spent melt from such molten salt combustion. The process results in maximizing the proportion of useful materials recovered from the molten salt combustion and minimizing the volume of material which must be discarded. In a preferred embodiment a spray dryer treatment is used to achieve the desired gas/liquid contact

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.

Waste package designs for disposal of high-level waste in salt formations

International Nuclear Information System (INIS)

Basham, S.J. Jr.; Carr, J.A.

1984-01-01

In the United States of America the selected method for disposal of radioactive waste is mined repositories located in suitable geohydrological settings. Currently four types of host rocks are under consideration: tuff, basalt, crystalline rock and salt. Development of waste package designs for incorporation in mined salt repositories is discussed. The three pertinent high-level waste forms are: spent fuel, as disassembled and close-packed fuel pins in a mild steel canister; commercial high-level waste (CHLW), as borosilicate glass in stainless-steel canisters; defence high-level waste (DHLW), as borosilicate glass in stainless-steel canisters. The canisters are production and handling items only. They have no planned long-term isolation function. Each waste form requires a different approach in package design. However, the general geometry and the materials of the three designs are identical. The selected waste package design is an overpack of low carbon steel with a welded closure. This container surrounds the waste forms. Studies to better define brine quantity and composition, radiation effects on the salt and brines, long-term corrosion behaviour of the low carbon steel, and the leaching behaviour of the spent fuel and borosilicate glass waste forms are continuing. (author)

Geologic disposal of nuclear wastes: salt's lead is challenged

International Nuclear Information System (INIS)

Kerr, R.A.

1979-01-01

The types of radioactive waste disposal sites available are outlined. The use of salt deposits and their advantages are discussed. The reasons for the selection of the present site for the Waste Isolation Pilot Plant are presented. The possibilities of using salt domes along the Gulf Coast and not-salt rocks as nuclear waste repositories are also discussed. The sea bed characteristics are described and advantages of this type of site selection are presented

Expected environment for waste packages in a salt repository

International Nuclear Information System (INIS)

Pederson, L.R.; Clark, D.E.; Hodges, F.N.; McVay, G.L.; Rai, D.

1983-01-01

This paper discusses results of recent efforts to define the very near-field (within approximately 2 m) environmental conditions to which waste packages will be exposed in a salt repository. These conditions must be considered in the experimental design for waste package materials testing, which includes corrosion of barrier materials and leaching of waste forms. Site-specific brine compositions have been determined, and standard brine compositions have been selected for testing purposes. Actual brine compositions will vary depending on origin, temperature, irradiation history, and contact with irradiated rock salt. Results of irradiating rock salt, synthetic brines, rock salt/brine mixtures, and reactions of irradiated rock salt with brine solutions are reported. 38 references, 3 figures, 2 tables

Molten salt processing of mixed wastes with offgas condensation

International Nuclear Information System (INIS)

Cooper, J.F.; Brummond, W.; Celeste, J.; Farmer, J.; Hoenig, C.; Krikorian, O.H.; Upadhye, R.; Gay, R.L.; Stewart, A.; Yosim, S.

1991-01-01

We are developing an advanced process for treatment of mixed wastes in molten salt media at temperatures of 700--1000 degrees C. Waste destruction has been demonstrated in a single stage oxidation process, with destruction efficiencies above 99.9999% for many waste categories. The molten salt provides a heat transfer medium, prevents thermal surges, and functions as an in situ scrubber to transform the acid-gas forming components of the waste into neutral salts and immobilizes potentially fugitive materials by a combination of particle wetting, encapsulation and chemical dissolution and solvation. Because the offgas is collected and assayed before release, and wastes containing toxic and radioactive materials are treated while immobilized in a condensed phase, the process avoids the problems sometimes associated with incineration processes. We are studying a potentially improved modification of this process, which treats oxidizable wastes in two stages: pyrolysis followed by catalyzed molten salt oxidation of the pyrolysis gases at ca. 700 degrees C. 15 refs., 5 figs., 1 tab

News about the genetics of congenital primary adrenal insufficiency.

Science.gov (United States)

Roucher-Boulez, Florence; Mallet-Motak, Delphine; Tardy-Guidollet, Véronique; Menassa, Rita; Goursaud, Claire; Plotton, Ingrid; Morel, Yves

2018-04-13

Primary adrenal insufficiency (PAI) is characterized by impaired production of steroid hormones due to an adrenal cortex defect. This condition incurs a risk of acute insufficiency which may be life-threatening. Today, 80% of pediatric forms of PAI have a genetic origin but 5% have no clear genetic support. Recently discovered mutations in genes relating to oxidative stress have opened the way to research on genes unrelated to the adrenal gland. Identification of causal mutations in a gene responsible for PAI allows genetic counseling, guidance of follow-up and prevention of complications. This is particularly true for stress oxidative anomalies, as extra-adrenal manifestations may occur due to the sensitivity to oxidative stress of other organs such as the heart, thyroid, liver, kidney and pancreas. Copyright © 2018 Elsevier Masson SAS. All rights reserved.

Radioactive waste and special waste disposal in salt domes - phoney waste management solutions

International Nuclear Information System (INIS)

Grimmel, E.

1990-01-01

The paper tries to make aware of the fact that an indefinite safe disposal of anthropogeneous wastes in underground repositories is impossible. Suspicion is raised that the Gorleben-Rambow salt dome has never been studied for its suitability as a repository, but that it was simply taken for granted. Safety analyses are meant only to conceal uncertainty. It is demanded to immediately opt out of the ultimate disposal technique for radioactive and special wastes in salt caverns. (DG) [de

Membrane Treatment of Liquid Salt Bearing Radioactive Wastes

International Nuclear Information System (INIS)

Dmitriev, S. A.; Adamovich, D. V.; Demkin, V. I.; Timofeev, E. M.

2003-01-01

The main fields of introduction and application of membrane methods for preliminary treatment and processing salt liquid radioactive waste (SLRW) can be nuclear power stations (NPP) and enterprises on atomic submarines (AS) utilization. Unlike the earlier developed technology for the liquid salt bearing radioactive waste decontamination and concentrating this report presents the new enhanced membrane technology for the liquid salt bearing radioactive waste processing based on the state-of-the-art membrane unit design, namely, the filtering units equipped with the metal-ceramic membranes of ''TruMem'' brand, as well as the electrodialysis and electroosmosis concentrators. Application of the above mentioned units in conjunction with the pulse pole changer will allow the marked increase of the radioactive waste concentrating factor and the significant reduction of the waste volume intended for conversion into monolith and disposal. Besides, the application of the electrodialysis units loaded with an ion exchange material at the end polishing stage of the radioactive waste decontamination process will allow the reagent-free radioactive waste treatment that meets the standards set for the release of the decontaminated liquid radioactive waste effluents into the natural reservoirs of fish-farming value

Immobilization of IFR salt wastes in mortar

International Nuclear Information System (INIS)

Fischer, D.F.; Johnson, T.R.

1988-01-01

Portland cement-base mortars are being considered for immobilizing chloride salt wastes produced by the fuel cycles of Integral Fast Reactors (IFR). The IFR is a sodium-cooled fast reactor with metal alloy fuels. It has a close-coupled fuel cycle in which fission products are separated from the actinides in an electrochemical cell operating at 500/degree/C. This cell has a liquid cadmium anode in which the fuels are dissolved and a liquid salt electrolyte. The salt will be a mixture of either lithium, potassium, and sodium chlorides or lithium, calcium, barium, and sodium chlorides. One method being considered for immobilizing the treated nontransuranic salt waste is to disperse the salt in a portland cement-base mortar that will be sealed in corrosion-resistant containers. For this application, the grout must be sufficiently fluid that it can be pumped into canister-molds where it will solidify into a strong, leach-resistant material. The set times must be longer than a few hours to allow sufficient time for processing, and the mortar must reach a reasonable compressive strength (/approximately/7 MPa) within three days to permit handling. Because fission product heating will be high, about 0.6 W/kg for a mortar containing 10% waste salt, the effects of elevated temperatures during curing and storage on mortar properties must be considered

Treatment of waste salts by oxygen sparging and vacuum distillation

International Nuclear Information System (INIS)

Cho, Y.J.; Yang, H.C.; Kim, E.H.; Kin, I.T.; Eun, H.C.

2007-01-01

Full text of publication follows. During the electrorefining process of the oxide spent fuel from LWR, amounts of waste salts containing some metal chloride species such as rare earths and actinide chlorides are generated, where the reuse of the waste salts is very important from the standpoint of an economical as well as an environmental aspect. In order to reuse the waste salts, a salt vacuum distillation method can be used. For the best separation by a vacuum distillation, the metal chloride species involved in the waste salts must be converted into their oxide(or oxychloride) forms due to the their low volatility compared to that of LiCl-KCl. In this study, an oxygen sparging process was adopted for the oxidation (or precipitation) of rare earth chlorides. The effects of oxygen flow rate and molten salt temperature on the conversion of rare earth chlorides to the precipitate phase (i.e. oxide or oxychloride) were investigated. In addition, distillation characteristics of LiCl-KCl molten salt with system pressure and temperature were studied. (authors)

Salt removal from tanks containing high-level radioactive waste

International Nuclear Information System (INIS)

Kiser, D.L.

1981-01-01

At the Savannah River Plant (SRP), there are 23 waste storage tanks containing high-level radioactive wastes that are to be retired. These tanks contain about 23 million liters of salt and about 10 million liters of sludge, that are to be relocated to new Type III, fully stress-relieved tanks with complete secondary containment. About 19 million liters of salt cake are to be dissolved. Steam jet circulators were originally proposed for the salt dissolution program. However, use of steam jet circulators raised the temperature of the tank contents and caused operating problems. These included increased corrosion risk and required long cooldown periods prior to transfer. Alternative dissolution concepts were investigated. Examination of mechanisms affecting salt dissolution showed that the ability of fresh water to contact the cake surface was the most significant factor influencing dissolution rate. Density driven and mechanical agitation techniques were developed on a bench scale and then were demonstrated in an actual waste tank. Actual waste tank demonstrations were in good agreement with bench-scale experiments at 1/85 scale. The density driven method utilizes simple equipment, but leaves a cake heel in the tank and is hindered by the presence of sludge or Zeolite in the salt cake. Mechanical agitation overcomes the problems found with both steam jet circulators and the density driven technique and is the best method for future waste tank salt removal

Risk assessment of nonhazardous oil-field waste disposal in salt caverns.

Energy Technology Data Exchange (ETDEWEB)

Elcock, D.

1998-03-10

Salt caverns can be formed in underground salt formations incidentally as a result of mining or intentionally to create underground chambers for product storage or waste disposal. For more than 50 years, salt caverns have been used to store hydrocarbon products. Recently, concerns over the costs and environmental effects of land disposal and incineration have sparked interest in using salt caverns for waste disposal. Countries using or considering using salt caverns for waste disposal include Canada (oil-production wastes), Mexico (purged sulfates from salt evaporators), Germany (contaminated soils and ashes), the United Kingdom (organic residues), and the Netherlands (brine purification wastes). In the US, industry and the regulatory community are pursuing the use of salt caverns for disposal of oil-field wastes. In 1988, the US Environmental Protection Agency (EPA) issued a regulatory determination exempting wastes generated during oil and gas exploration and production (oil-field wastes) from federal hazardous waste regulations--even though such wastes may contain hazardous constituents. At the same time, EPA urged states to tighten their oil-field waste management regulations. The resulting restrictions have generated industry interest in the use of salt caverns for potentially economical and environmentally safe oil-field waste disposal. Before the practice can be implemented commercially, however, regulators need assurance that disposing of oil-field wastes in salt caverns is technically and legally feasible and that potential health effects associated with the practice are acceptable. In 1996, Argonne National Laboratory (ANL) conducted a preliminary technical and legal evaluation of disposing of nonhazardous oil-field wastes (NOW) into salt caverns. It investigated regulatory issues; the types of oil-field wastes suitable for cavern disposal; cavern design and location considerations; and disposal operations, closure and remediation issues. It determined

Risk assessment of nonhazardous oil-field waste disposal in salt caverns

International Nuclear Information System (INIS)

Elcock, D.

1998-01-01

Salt caverns can be formed in underground salt formations incidentally as a result of mining or intentionally to create underground chambers for product storage or waste disposal. For more than 50 years, salt caverns have been used to store hydrocarbon products. Recently, concerns over the costs and environmental effects of land disposal and incineration have sparked interest in using salt caverns for waste disposal. Countries using or considering using salt caverns for waste disposal include Canada (oil-production wastes), Mexico (purged sulfates from salt evaporators), Germany (contaminated soils and ashes), the United Kingdom (organic residues), and the Netherlands (brine purification wastes). In the US, industry and the regulatory community are pursuing the use of salt caverns for disposal of oil-field wastes. In 1988, the US Environmental Protection Agency (EPA) issued a regulatory determination exempting wastes generated during oil and gas exploration and production (oil-field wastes) from federal hazardous waste regulations--even though such wastes may contain hazardous constituents. At the same time, EPA urged states to tighten their oil-field waste management regulations. The resulting restrictions have generated industry interest in the use of salt caverns for potentially economical and environmentally safe oil-field waste disposal. Before the practice can be implemented commercially, however, regulators need assurance that disposing of oil-field wastes in salt caverns is technically and legally feasible and that potential health effects associated with the practice are acceptable. In 1996, Argonne National Laboratory (ANL) conducted a preliminary technical and legal evaluation of disposing of nonhazardous oil-field wastes (NOW) into salt caverns. It investigated regulatory issues; the types of oil-field wastes suitable for cavern disposal; cavern design and location considerations; and disposal operations, closure and remediation issues. It determined

Steroid abnormalities and the developing brain: Declarative memory for emotionally arousing and neutral material in children with congenital adrenal hyperplasia

OpenAIRE

Maheu, Françoise S.; Merke, Deborah P.; Schroth, Elizabeth A.; Keil, Margaret F.; Hardin, Julie; Poeth, Kaitlin; Pine, Daniel S.; Ernst, Monique

2007-01-01

Steroid hormones modulate memory in animals and human adults. Little is known on the developmental effect of these hormones on the neural networks underlying memory. Using Congenital Adrenal Hyperplasia (CAH) as a naturalistic model of early steroid abnormalities, this study examines the consequences of CAH on memory and its neural correlates for emotionally arousing and neutral material in children. Seventeen patients with CAH and 17 age- and sex-matched healthy children (ages 12 to 14 years...

Saltstone: cement-based waste form for disposal of Savannah River Plant low-level radioactive salt waste

International Nuclear Information System (INIS)

Langton, C.A.

1984-01-01

Defense waste processing at the Savannah River Plant will include decontamination and disposal of approximately 400 million liters of waste containing NaNO 3 , NaOH, Na 2 SO 4 , and NaNO 2 . After decontamination, the salt solution is classified as low-level waste. A cement-based waste form, saltstone, has been designed for disposal of Savannah River Plant low-level radioactive salt waste. Bulk properties of this material have been tailored with respect to salt leach rate, permeability, and compressive strength. Microstructure and mineralogy of leached and unleached specimens were characterized by SEM and x-ray diffraction analyses. The disposal system for the DWPF salt waste includes reconstitution of the crystallized salt as a solution containing 32 wt % solids. This solution will be decontaminated to remove 137 Cs and 90 Sr and then stabilized in a cement-based waste form. Laboratory and field tests indicate that this stabilization process greatly reduces the mobility of all of the waste constitutents in the surface and near-surface environment. Engineered trenches for subsurface burial of the saltstone have been designed to ensure compatibility between the waste form and the environment. The total disposal sytem, saltstone-trench-surrounding soil, has been designed to contain radionuclides, Cr, and Hg by both physical encapsulation and chemical fixation mechanisms. Physical encapsulation of the salts is the mechanism employed for controlling N and OH releases. In this way, final disposal of the SRP low-level waste can be achieved and the quality of the groundwater at the perimeter of the disposal site meets EPA drinking water standards

High-temperature vacuum distillation separation of plutonium waste salts

International Nuclear Information System (INIS)

Garcia, E.

1996-01-01

In this task, high-temperature vacuum distillation separation is being developed for residue sodium chloride-potassium chloride salts resulting from past pyrochemical processing of plutonium. This process has the potential of providing clean separation of the salt and the actinides with minimal amounts of secondary waste generation. The process could produce chloride salt that could be discarded as low-level waste (LLW) or low actinide content transuranic (TRU) waste, and a concentrated actinide oxide powder that would meet long-term storage standards (DOE-DTD-3013-94) until a final disposition option for all surplus plutonium is chosen

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.

Stabilization Using Phosphate Bonded Ceramics. Salt Containing Mixed Waste Treatment. Mixed Waste Focus Area. OST Reference No. 117

International Nuclear Information System (INIS)

1999-01-01

Throughout the Department of Energy (DOE) complex there are large inventories of homogeneous mixed waste solids, such as wastewater treatment residues, fly ashes, and sludges that contain relatively high concentrations (greater than 15% by weight) of salts. The inherent solubility of salts (e.g., nitrates, chlorides, and sulfates) makes traditional treatment of these waste streams difficult, expensive, and challenging. One alternative is low-temperature stabilization by chemically bonded phosphate ceramics (CBPCs). The process involves reacting magnesium oxide with monopotassium phosphate with the salt waste to produce a dense monolith. The ceramic makes a strong environmental barrier, and the metals are converted to insoluble, low-leaching phosphate salts. The process has been tested on a variety of surrogates and actual mixed waste streams, including soils, wastewater, flyashes, and crushed debris. It has also been demonstrated at scales ranging from 5 to 55 gallons. In some applications, the CBPC technology provides higher waste loadings and a more durable salt waste form than the baseline method of cementitious grouting. Waste form test specimens were subjected to a variety of performance tests. Results of waste form performance testing concluded that CBPC forms made with salt wastes meet or exceed both RCRA and recommended Nuclear Regulatory Commission (NRC) low-level waste (LLW) disposal criteria. Application of a polymer coating to the CBPC may decrease the leaching of salt anions, but continued waste form evaluations are needed to fully assess the deteriorating effects of this leaching, if any, over time.

Treatment of waste salt from the advanced spent fuel conditioning process (I): characterization of Zeolite A in Molten LiCl Salt

International Nuclear Information System (INIS)

Kim, Jeong Guk; Lee, Jae Hee; Yoo, Jae Hyung; Kim, Joon Hyung

2004-01-01

The oxide fuel reduction process based on the electrochemical method (Advanced spent fuel Conditioning Process; ACP) and the long-lived radioactive nuclides partitioning process based on electro-refining process, which are being developed ay the Korea Atomic Energy Research Institute (KAERI), are to generate two types of molten salt wastes such as LiCl salt and LiCl-KCl eutectic salt, respectively. These waste salts must meet some criteria for disposal. A conditioning process for LiCl salt waste from ACP has been developed using zeolite A. This treatment process of waste salt using zeolite A was first developed by US ANL (Argonne National Laboratory) for LiCl-KCl eutectic salt waste from an electro-refining process of EBR (Experimental Breeder Reactor)-II spent fuel. This process has been developed recently, and a ceramic waste form (CWF) is produced in demonstration-scale V-mixer (50 kg/batch). However, ANL process is different from KAERI treatment process in waste salt, the former is LiCl-KCl eutectic salt and the latter is LiCl salt. Because of melting point, the immobilization of eutectic salt is carried out at about 770 K, whereas LiCl salt at around 920 K. Such difference has an effect on properties of immobilization media, zeolite A. Here, zeolite A in high-temperature (923 K) molten LiCl salt was characterized by XRD, Ion-exchange, etc., and evaluated if a promising media or not

Expected brine movement at potential nuclear waste repository salt sites

International Nuclear Information System (INIS)

McCauley, V.S.; Raines, G.E.

1987-08-01

The BRINEMIG brine migration code predicts rates and quantities of brine migration to a waste package emplaced in a high-level nuclear waste repository in salt. The BRINEMIG code is an explicit time-marching finite-difference code that solves a mass balance equation and uses the Jenks equation to predict velocities of brine migration. Predictions were made for the seven potentially acceptable salt sites under consideration as locations for the first US high-level nuclear waste repository. Predicted total quantities of accumulated brine were on the order of 1 m 3 brine per waste package or less. Less brine accumulation is expected at domal salt sites because of the lower initial moisture contents relative to bedded salt sites. Less total accumulation of brine is predicted for spent fuel than for commercial high-level waste because of the lower temperatures generated by spent fuel. 11 refs., 36 figs., 29 tabs

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.

Criticality considerations for salt-cake disolution in DOE waste tanks

International Nuclear Information System (INIS)

Trumble, E.F.; Niemer, K.A.

1995-01-01

A large amount of high-level waste is being stored in the form of salt cake at the Savannah River site (SRS) in large (1.3 x 106 gal) underground tanks awaiting startup of the Defense Waste Processing Facility (DWPF). This salt cake will be dissolved with water, and the solution will be fed to DWPF for immobilization in borosilicate glass. Some of the waste that was transferred to the tanks contained enriched uranium and plutonium from chemical reprocessing streams. As water is added to these tanks to dissolve the salt cake, the insoluble portion of this fissile material will be left behind in the tank as the salt solution is pumped out. Because the salt acts as a diluent to the fissile material, the process of repeated water addition, salt dissolution, and salt solution removal will act as a concentrating mechanism for the undissolved fissile material that will remain in the tank. It is estimated that tank 41 H at SRS contains 20 to 120 kg of enriched uranium, varying from 10 to 70% 235 U, distributed nonuniformly throughout the tank. This paper discusses the criticality concerns associated with the dissolution of salt cake in this tank. These concerns are also applicable to other salt cake waste tanks that contain significant quantities of enriched uranium and/or plutonium

Release rates from waste packages in a salt repository

International Nuclear Information System (INIS)

Chambre, P.L.; Hwang, Y.; Lee, W.W.L.; Pigford, T.H.

1987-06-01

In this report we present estimates of radionuclide release rates from waste packages into salt. This conservative and bounding analysis shows that release rates from waste packages in salt are well below the US Nuclear Regulatory Commission's performance objectives for the engineered barrier system. 2 refs., 2 figs

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

Directory of Open Access Journals (Sweden)

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.

Laboratory simulation of salt dissolution during waste removal

International Nuclear Information System (INIS)

Wiersma, B.J.; Parish, W.R.

1997-01-01

Laboratory experiments were performed to support the field demonstration of improved techniques for salt dissolution in waste tanks at the Savannah River Site. The tests were designed to investigate three density driven techniques for salt dissolution: (1) Drain-Add-Sit-Remove, (2) Modified Density Gradient, and (3) Continuous Salt Mining. Salt dissolution was observed to be a very rapid process as salt solutions with densities between 1.38-1.4 were frequently removed. Slower addition and removal rates and locating the outlet line at deeper levels below the top of the saltcake provided the best contact between the dissolution water and the saltcake. It was observed that dissolution with 1 M sodium hydroxide solution resulted in salt solutions that were within the current inhibitor requirements for the prevention of stress corrosion cracking. This result was independent of the density driven technique. However, if inhibited water (0.01 M sodium hydroxide and 0.011 M sodium nitrite) was utilized, the salt solutions were frequently outside the inhibitor requirements. Corrosion testing at conditions similar to the environments expected during waste removal was recommended

The safe disposal of radioactive wastes in geologic salt formations

International Nuclear Information System (INIS)

Kuehn, K.; Proske, R.

Geologic salt formations appear to be particularly suitable for final storage. Their existance alone - the salt formations in Northern Germany are more than 200 million years old - is proof of their stability and of their isolation from biological cycles. In 1967 the storage of LAW and later, in 1972, of MAW was started in the experimental storage area Asse, south-east of Braunschweig, after the necessary technical preparations had been made. In more than ten years of operation approx. 114,000 drums of slightly active and 1,298 drums of medium-active wastes were deposited without incident. Methods have been developed for filling the available caverns with wastes and salt to ensure the security of long term disposal without supervision. Tests with electric heaters for simulation of heat-generating highly active wastes confirm the good suitability of salt formations for storing these wastes. Safety analyses for the operating time as well as for the long term phase after closure of the final storage area, which among others also comprise the improbable ''greatest expected accident'', namely break through of water, are carried out and confirm the safety of ultimate storage of radioactive wastes in geological salt formations. (orig./HP) [de

Pubertal development among girls with classical congenital adrenal hyperplasia initiated on treatment at different ages

Directory of Open Access Journals (Sweden)

Bindu Kulshreshtha

2012-01-01

Full Text Available Introduction: Children with congenital adrenal hyperplasia (CAH provide us an opportunity to study the clinical effects of androgen excess in humans. We studied the sequence of pubertal development in girls with congenital adrenal hyperplasia initiated on treatment at different ages, to assess the effects of androgen exposure on the Hypothalamic-Pituitary-Ovarian (HPO axis. Materials and Methods: Girls more than 18 years of age, with CAH, on follow-up at this hospital were the subjects for this study. Details of history, physical findings, laboratory evaluation, and medication were noted from their case records and verified from the patients and their / parents, in addition to assessment of their present health status. Result: We studied 24 patients of classical CAH (SW-2, SV-22, average age - 24.5 ± 6.6 years. All had varying degrees of genital ambiguity (Prader stage 3 (n = 13, Prader stage 2 (n = 10, Prader stage 1 (n = 1. Among them were13 girls, who were started on steroids after eight years of age. Girls who received treatment from infancy and early childhood had normal pubertal development (mean age at menarche 11.4 ± 1.7 years. Hirsutism was not a problem among them. Untreated children had progressive clitoral enlargement throughout childhood, developed pubic hair at around three to six years of age, and facial hair between nine and eleven years. Plasma testosterone ranged from 3 to 6 ng / ml prior to treatment. Six of the 13 untreated CAH girls had subtle breast development starting at ages 11 - 16 years and three had spontaneous infrequent vaginal bleeding starting at ages 11 - 17. Steroid supplementation initiated pubertal changes in older girls in two-to-six months′ time. Conclusion: There was a delay in HPO axis maturation (as evidenced by delayed pubertal development in the absence of treatment in girls with CAH. This could be corrected with steroid supplementation.

Alternative methods of salt disposal at the seven salt sites for a nuclear waste repository

International Nuclear Information System (INIS)

1987-02-01

This study discusses the various alternative salt management techniques for the disposal of excess mined salt at seven potentially acceptable nuclear waste repository sites: Deaf Smith and Swisher Counties, Texas; Richton and Cypress Creek Domes, Mississippi; Vacherie Dome, Louisiana; and Davis and Lavender Canyons, Utah. Because the repository development involves the underground excavation of corridors and waste emplacement rooms, in either bedded or domed salt formations, excess salt will be mined and must be disposed of offsite. The salt disposal alternatives examined for all the sites include commercial use, ocean disposal, deep well injection, landfill disposal, and underground mine disposal. These alternatives (and other site-specific disposal methods) are reviewed, using estimated amounts of excavated, backfilled, and excess salt. Methods of transporting the excess salt are discussed, along with possible impacts of each disposal method and potential regulatory requirements. A preferred method of disposal is recommended for each potentially acceptable repository site. 14 refs., 5 tabs

Waste management analysis for the nuclear fuel cycle. I. Actinide recovery from aqueous salt wastes

International Nuclear Information System (INIS)

Martella, L.L.; Navratil, J.D.

1979-01-01

A preliminary feasibility study of solvent extraction methods has been completed for removing actinides from selected salt wastes likely to be produced during reactor fuel fabrication and reprocessing. The use of a two-step solvent extraction system, tributyl phosphate (TBP) followed by a bidentate organophosphorus extractant (DHDECMP), appears most efficient for removing actinides from salt waste. The TBP step would remove most of the plutonium and >99.99% of the uranium. The second step, using DHDECMP, would remove >99.91% of the americium, the remaining plutonium (>99.98%), and other actinides from the acidified salt waste

Defense waste salt disposal at the Savannah River Plant

International Nuclear Information System (INIS)

Langton, C.A.; Dukes, M.D.

1984-01-01

A cement-based waste form, saltstone, has been designed for disposal of Savannah River Plant low-level radioactive salt waste. The disposal process includes emplacing the saltstone in engineered trenches above the water table but below grade at SRP. Design of the waste form and disposal system limits the concentration of salts and radionuclides in the groundwater so that EPA drinking water standards will not be exceeded at the perimeter of the disposal site. 10 references, 4 figures, 3 tables

Expedited demonstration of molten salt mixed waste treatment technology. Final report

International Nuclear Information System (INIS)

1995-01-01

This final report discusses the molten salt mixed waste project in terms of the various subtasks established. Subtask 1: Carbon monoxide emissions; Establish a salt recycle schedule and/or a strategy for off-gas control for MWMF that keeps carbon monoxide emission below 100 ppm on an hourly averaged basis. Subtask 2: Salt melt viscosity; Experiments are conducted to determine salt viscosity as a function of ash composition, ash concentration, temperature, and time. Subtask 3: Determine that the amount of sodium carbonate entrained in the off-gas is minimal, and that any deposited salt can easily be removed form the piping using a soot blower or other means. Subtask 4: The provision of at least one final waste form that meets the waste acceptance criteria of a landfill that will take the waste. This report discusses the progress made in each of these areas

A case of adrenal Cushing's syndrome with bilateral adrenal masses.

Science.gov (United States)

Guo, Ya-Wun; Hwu, Chii-Min; Won, Justin Ging-Shing; Chu, Chia-Huei; Lin, Liang-Yu

2016-01-01

A functional lesion in corticotrophin (ACTH)-independent Cushing's syndrome is difficult to distinguish from lesions of bilateral adrenal masses. Methods for distinguishing these lesions include adrenal venous sampling and (131)I-6β-iodomethyl-19-norcholesterol ((131)I-NP-59) scintigraphy. We present a case of a 29-year-old Han Chinese female patient with a history of hypercholesterolaemia and polycystic ovary syndrome. She presented with a 6month history of an 8kg body weight gain and gradual rounding of the face. Serial examinations revealed loss of circadian rhythm of cortisol, elevated urinary free-cortisol level and undetectable ACTH level (Cushing's syndrome presenting with bilateral adrenal masses. The clinical presentation of Cushing' syndrome includes symptoms and signs of fat redistribution and protein-wasting features.The diagnosis of patients with ACTH-independent Cushing's syndrome with bilateral adrenal masses is challenging for localisation of the lesion.Both adrenal venous sampling and (131)I-NP-59 scintigraphy are good methods to use in these patients with Cushing's syndrome presenting with bilateral adrenal masses.

Salt Repository Project Waste Package Program Plan: Draft

International Nuclear Information System (INIS)

Carr, J.A.; Cunnane, J.C.

1986-01-01

Under the direction of the Office of Civilian Radioactive Waste Management (OCRWM) created within the DOE by direction of the Nuclear Waste Policy Act of 1982 (NWPA), the mission of the Salt Repository Project (SRP) is to provide for the development of a candidate salt repository for disposal of high-level radioactive waste (HLW) and spent reactor fuel in a manner that fully protects the health and safety of the public and the quality of the environment. In consideration of the program needs and requirements discussed above, the SRP has decided to develop and issue this SRP Waste Package Program Plan. This document is intended to outline how the SRP plans to develop the waste package design and to show, with reasonable assurance, that the developed design will satisfy applicable requirements/performance objectives. 44 refs., 16 figs., 16 tabs

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.

Salt splitting of sodium-dominated radioactive waste using ceramic membranes

International Nuclear Information System (INIS)

Hollenberg, G.W.; Carlson, C.D.; Virkar, A.; Joshi, A.

1994-08-01

The potential for salt splitting of sodium dominated radioactive wastes by use of a ceramic membrane is reviewed. The technical basis for considering this processing technology is derived from the technology developed for battery and chlor-alkali chemical industry. Specific comparisons are made with the commercial organic membranes which are the standard in nonradioactive salt splitting. Two features of ceramic membranes are expected to be especially attractive: high tolerance to gamma irradiation and high selectivity between sodium and other ions. The objective of the salt splitting process is to separate nonradioactive sodium from contaminated sodium salts prior to other pretreatment processes in order to: (1) concentrate the waste in order to reduce the volume of subsequent additives and capacity of equipment, (2) decrease the pH of the waste in preparation for further processing, and (3) provide sodium with very low radioactivity levels for caustic washing of sludge or low level and mixed waste vitrification

Molten salt destruction process for mixed wastes

International Nuclear Information System (INIS)

Upadhye, R.S.; Wilder, J.G.; Karlsen, C.E.

1993-04-01

We are developing an advanced two-stage process for the treatment of mixed wastes, which contain both hazardous and radioactive components. The wastes, together with an oxidant gas, such as air, are injected into a bed of molten salt comprising a mixture of sodium-, potassium-, and lithium-carbonates, with a melting point of about 580 degree C. The organic constituents of the mixed waste are destroyed through the combined effect of pyrolysis and oxidation. Heteroatoms. such as chlorine, in the mixed waste form stable salts, such as sodium chloride, and are retained in the melt. The radioactive actinides in the mixed waste are also retained in the melt because of the combined action of wetting and partial dissolution. The original process, consists of a one-stage unit, operated at 900--1000 degree C. The advanced two-stage process has two stages, one for pyrolysis and one for oxidation. The pyrolysis stage is designed to operate at 700 degree C. The oxidation stage can be operated at a higher temperature, if necessary

[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.

R and D activities on the management of waste chloride salts in KAERI

International Nuclear Information System (INIS)

In-Tae, Kim; Hwan-Seo, Park; Jeong-Gook, Kim; Hee-Chul, Yang; Yong-Joon, Cho; Eung-Ho Kim

2007-01-01

Full text of publication follows. Electrochemical treatment of spent oxide fuels has been intensively studied in KAERI to reduce the volume, heat load and radiotoxicity of high-level wastes. It consists of an electrolytic reduction process to convert the oxide fuel into a metallic form and an electro-refining process to separate TRU elements from the electro-reduced metal ingot. Two types of waste salts are expected to generate from the electrochemical pyro-processes, that is, LiCl salt from the reduction process and LiCl+KCl eutectic salt form the refining process. The R and D strategy of the waste salt management in KAERI can be categorized into two parts: 1) enhancement of safety by the stabilisation/solidification of waste salt that is to be finally disposed of and 2) reduction of the waste generation by the regeneration/recycle of the spent salt after removal of radionuclides in it. A sol-gel technique and a zeolite occlusion technique are under development to stabilize the waste salt. The LiCl salt is stabilised by a low-temperature sol-gel process and then the gel product is solidified into a ceramic-like waste form with an addition of glass frit. Another method uses Zeolite-4A to occlude the LiCl salt into its cage and adsorption site to immobilize the radionuclides. The product, salt-occluded zeolite, is fabricated into another type of a ceramic waste form. For the regeneration and recycle of the spent salt, the radionuclides in the salt are removed by a zeolite process for the LiCl salt and by an oxidation/distillation process for the eutectic salt. The target nuclides to be removed in each process are Cs/Sr and rare earth (RE) elements, respectively. In the oxidation/ distillation process, the rare earth chloride nuclides are oxidised by an oxygen sparging method, and the products are precipitated in the form of oxide or oxychloride REs. After separation of the RE elements from the precipitates by distillation, the refined spent salt with a low content

Containment of solidified liquid hazardous waste in domal salt

International Nuclear Information System (INIS)

Domenico, P.A.; Lerman, A.

1992-01-01

In recent years, the solidification of hazardous liquid waste has become a viable option in waste management. The solidification process results in an increased volume but more stable waste form that must be disposed of or stored in a dry environment. An environment of choice in south central Texas is domal salt. The salt dome currently under investigation has a water content of 0.002 percent by weight and a permeability less than one nanodarcy. A question that must be addressed is whether a salt dome has a particular set of attributes that will prevent the release of contaminants to the environment. From a regulatory perspective, a ''no migration'' petition must be approved by the U.S.E.P.A. for the containment facility. By ''no migration'' it is implied that the waste must be contained for 10,000 years. A demonstration that this condition will be met will require model calculations and such models must be based on the physical and chemical characteristics of the waste form and the geologic environment. In particular, the models must address the rate of brine infiltration into the caverns, providing information on how fast an immobile solid waste form could convert to a more mobile liquid state. Additionally, the potential for migration by both diffusion and advection is of concern. Lastly, given a partially saturated cavern, the question of how far gaseous waste will be transported over the 10,000 year containment period must also be addressed. Results indicate that the containment capabilities of domal salt are exceptional. A nominal volume of brine will seep into the cavern and most voids between the injected solidified waste pellets will remain unsaturated. Very small quantities of hazardous constituents will be leached from the waste pellets

Children with classic congenital adrenal hyperplasia have elevated serum leptin concentrations and insulin resistance: potential clinical implications.

Science.gov (United States)

Charmandari, Evangelia; Weise, Martina; Bornstein, Stefan R; Eisenhofer, Graeme; Keil, Margaret F; Chrousos, George P; Merke, Deborah P

2002-05-01

Leptin is secreted by the white adipose tissue and modulates energy homeostasis. Nutritional, neural, neuroendocrine, paracrine, and autocrine factors, including the sympathetic nervous system and the adrenal medulla, have been implicated in the regulation of leptin secretion. Classic congenital adrenal hyperplasia (CAH) is characterized by a defect in cortisol and aldosterone secretion, impaired development and function of the adrenal medulla, and adrenal hyperandrogenism. To examine leptin secretion in patients with classic CAH in relation to their adrenomedullary function and insulin and androgen secretion, we studied 18 children with classic CAH (12 boys and 6 girls; age range 2-12 yr) and 28 normal children (16 boys and 12 girls; age range 5-12 yr) matched for body mass index (BMI). Serum leptin concentrations were significantly higher in patients with CAH than in control subjects (8.1 +/- 2.0 vs. 2.5 +/- 0.6 ng/ml, P = 0.01), and this difference persisted when leptin values were corrected for BMI. When compared with their normal counterparts, children with CAH had significantly lower plasma epinephrine (7.1 +/- 1.3 vs. 50.0 +/- 4.2, P fasting serum insulin (10.6 +/- 1.4 vs. 3.2 +/- 0.2 microU/ml, P Insulin resistance determined by the homeostasis model assessment method was significantly greater in children with classic CAH than in normal children (2.2 +/- 0.3 vs. 0.7 +/- 0.04, P patients and controls. Gender predicted serum leptin concentrations in controls but not in patients with classic CAH. No association was found between the dose of hydrocortisone and serum leptin (r = -0.17, P = 0.5) or insulin (r = 0.24, P = 0.3) concentrations in children with CAH. Our findings indicate that children with classic CAH have elevated fasting serum leptin and insulin concentrations, and insulin resistance. These most likely reflect differences in long-term adrenomedullary hypofunction and glucocorticoid therapy. Elevated leptin and insulin concentrations in patients

Congenital adrenal hyperplasia: as viewed by parents of affected children in India--a pilot study.

Science.gov (United States)

Bhakhri, Bhanu Kiran; Jain, Vandana

2011-01-01

A spectrum of myths and misconceptions about congenital adrenal hyperplasia (CAH) is prevalent among the parents of affected children in India. The perceptions of parents may affect several aspects of these children's management, and to explore these perceptions we carried out a cross-sectional questionnaire-based descriptive study during May 2010. Twenty-eight individuals (17 males and 11 females), parents of 22 affected children aged parents of misconceptions about CAH. These misconceptions were resulting in potentially harmful practices, and in addition there was immense societal pressure on the families as a result of ignorance and myths about the disorder. There is a need for regular CAH education and interaction programs to provide an acceptable platform for the parents and patients, where their concerns can be expressed and shared and their requirements addressed appropriately by a multidisciplinary team.

Characteristics of solidified products containing radioactive molten salt waste.

Science.gov (United States)

Park, Hwan-Seo; Kim, In-Tae; Cho, Yong-Zun; Eun, Hee-Chul; Kim, Joon-Hyung

2007-11-01

The molten salt waste from a pyroprocess to recover uranium and transuranic elements is one of the problematic radioactive wastes to be solidified into a durable wasteform for its final disposal. By using a novel method, named as the GRSS (gel-route stabilization/solidification) method, a molten salt waste was treated to produce a unique wasteform. A borosilicate glass as a chemical binder dissolves the silicate compounds in the gel products to produce one amorphous phase while most of the phosphates are encapsulated by the vitrified phase. Also, Cs in the gel product is preferentially situated in the silicate phase, and it is vitrified into a glassy phase after a heat treatment. The Sr-containing phase is mainly phosphate compounds and encapsulated by the glassy phase. These phenomena could be identified by the static and dynamic leaching test that revealed a high leach resistance of radionuclides. The leach rates were about 10(-3) - 10(-2) g/m2 x day for Cs and 10(-4) - 10(-3) g/m2 x day for Sr, and the leached fractions of them were predicted to be 0.89% and 0.39% at 900 days, respectively. This paper describes the characteristics of a unique wasteform containing a molten salt waste and provides important information on a newly developed immobilization technology for salt wastes, the GRSS method.

Problems of the final storage of radioactive waste in salt formations

International Nuclear Information System (INIS)

Hofrichter, E.

1977-01-01

The geological conditions for the final storage of radioactive waste, the occurrence of salt formations, and the tectonics of salt domes are discussed. The safety of salt rocks, the impermeability of the rocks, and the thermal problems in the storage of high-activity waste are dealt with. Possibilities and preconditions of final storage in West Germany are discussed. (HPH) [de

Thermal denitration of high concentration nitrate salts waste water

International Nuclear Information System (INIS)

Hwang, D. S.; Oh, J. H.; Choi, Y. D.; Hwang, S. T.; Park, J. H.; Latge, C.

2003-01-01

This study investigated the thermodynamic and the thermal decomposition properties of high concentration nitrate salts waste water for the lagoon sludge treatment. The thermodynamic property was carried out by COACH and GEMINI II based on the composition of nitrate salts waste water. The thermal decomposition property was carried out by TG-DTA and XRD. Ammonium nitrate and sodium nitrate were decomposed at 250 .deg. C and 730 . deg. C, respectively. Sodium nitrate could be decomposed at 450 .deg. C in the case of adding alumina for converting unstable Na 2 O into stable Na 2 O.Al 2 O 3 . The flow sheet for nitrate salts waste water treatment was proposed based on the these properties data. These will be used by the basic data of the process simulation

Method to synthesize dense crystallized sodalite pellet for immobilizing halide salt radioactive waste

International Nuclear Information System (INIS)

Koyama, Tadafumi.

1994-01-01

A method is described for immobilizing waste chloride salts containing radionuclides such as cesium and strontium and hazardous materials such as barium. A sodalite intermediate is prepared by mixing appropriate amounts of silica, alumina and sodium hydroxide with respect to sodalite and heating the mixture to form the sodalite intermediate and water. Heating is continued to drive off the water to form a water-free intermediate. The water-free intermediate is mixed with either waste salt or waste salt which has been contacted with zeolite to concentrate the radionuclides and hazardous material. The waste salt-intermediate mixture is then compacted and heated under conditions of heat and pressure to form sodalite with the waste salt, radionuclides and hazardous material trapped within the sodalite cage structure. This provides a final product having excellent leach resistant capabilities

Embryology of the adrenal glands and its relevance to diagnostic imaging

International Nuclear Information System (INIS)

Barwick, T.D.; Malhotra, A.; Webb, J.A.W.; Savage, M.O.; Reznek, R.H.

2005-01-01

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

Congenital bilateral neuroblastoma (stage IV-S): case report

International Nuclear Information System (INIS)

Lee, Jeong Hee; Lee, Hee Jung; Woo, Seong Ku; Lee, Sang Rak; Kim, Heung Sik

2002-01-01

Congenital neonatal neuroblastoma is not uncommon but bilateral adrenal neuroblastoma is rare, accounting for about ten percent of neuroblastomas in children. We report the US the MR findings of a stage IV-S congenital bilateral neuroblastoma occurring in a one-day-old neonate

Stigma Associated with Classical Congenital Adrenal Hyperplasia in Women's Sexual Lives.

Science.gov (United States)

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

2018-05-01

The risk of intersex-related stigma often serves as social indication for "corrective" genital surgery, but has not been comprehensively documented. In preparation for the development of an intersex-specific stigma assessment tool, this qualitative project aimed to explore stigma in girls and women with classical congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. As part of a comprehensive follow-up project, 62 adult women with classical CAH (age range 18-51 years) took part in an open-ended retrospective interview focusing on the impact of CAH and its treatment on various aspects of girls' and women's lives. Deductive qualitative content analysis (Patton, 2014) of de-identified transcripts involved categorization of three types of stigma: experienced, anticipated, and internalized. Two-fifths of the participants reported CAH-related stigma in romantic/sexual situations. Stigma enactment by romantic partners occurred in reaction to both genital and non-genital sex-atypical features of CAH and sometimes included explicit questioning of the women's true gender. Stigma anticipation by the women and their related avoidance of nudity, genital exposure, and romantic involvement altogether were frequent. Internalization of stigma occurred as well. In conclusion, the data suggest that many women with CAH experience, anticipate, and/or internalize intersex-related stigma in the context of their romantic/sexual lives.

Motor development in individuals with congenital adrenal hyperplasia: strength, targeting, and fine motor skill.

Science.gov (United States)

Collaer, Marcia L; Brook, Charles G D; Conway, Gerard S; Hindmarsh, Peter C; Hines, Melissa

2009-02-01

This study investigated early androgen influence on the development of human motor and visuomotor characteristics. Participants, ages 12-45 years, were individuals with congenital adrenal hyperplasia (CAH), a disorder causing increased adrenal androgen production before birth (40 females, 29 males) and their unaffected relatives (29 females, 30 males). We investigated grip strength and visuomotor targeting tasks on which males generally outperform females, and fine motor pegboard tasks on which females generally outperform males. Physical characteristics (height and weight) were measured to explore whether body parameters could explain differences in motor skills. Females with CAH were stronger and showed better targeting than unaffected females and showed reduced fine visuomotor skill on one pegboard measure, with no difference on the other. Males with CAH were weaker than unaffected males in grip strength but did not differ on the targeting or pegboard measures. Correction for body size could not explain the findings for females, but suggests that the reduced strength of males with CAH may relate to their smaller stature. Further, the targeting advantage in females with CAH persisted following adjustment for their greater strength. Results in females support the hypothesis that androgen may masculinize, or promote, certain motor characteristics at which males excel, and contribute to defeminization of certain fine motor characteristics at which females excel. Thus, these data suggest that organizational effects of androgens on behavior during prenatal life may extend to motor characteristics and may contribute to general sex differences in motor-related behaviors; however, alternative explanations based on activational influences of androgen or altered experiential factors cannot be excluded without further study.

Assessment of crushed salt consolidation and fracture healing processes in a nuclear waste repository in salt

International Nuclear Information System (INIS)

1984-11-01

For a nuclear waste repository in salt, two aspects of salt behavior are expected to contribute to favorable conditions for waste isolation. First, consolidation of crushed salt backfill due to creep closure of the underground openings may result in a backfill barrier with low permeability. Second, fractures created in the salt by excavation may heal under the influence of stress and temperature following sealing. This report reviews the status of knowledge regarding crushed salt consolidation and fracture healing, provides analyses which predict the rates at which the processes will occur under repository conditions, and develops requirements for future study. Analyses of the rate at which crushed salt will consolidate are found to be uncertain because of unexplained wide variation in the creep properties of crushed salt obtained from laboratory testing, and because of uncertainties in predictions of long term closure rates of openings in salt. This uncertainty could be resolved to a large degree by additional laboratory testing of crushed salt. Similarly, additional testing of fracture healing processes is required to confirm that healing will be effective under repository conditions. Extensive references, 27 figures, 5 tables

Systems costs for disposal of Savannah River high-level waste sludge and salt

International Nuclear Information System (INIS)

McDonell, W.R.; Goodlett, C.B.

1984-01-01

A systems cost model has been developed to support disposal of defense high-level waste sludge and salt generated at the Savannah River Plant. Waste processing activities covered by the model include decontamination of the salt by a precipitation process in the waste storage tanks, incorporation of the sludge and radionuclides removed from the salt into glass in the Defense Waste Processing Facility (DWPF), and, after interim storage, final disposal of the DWPF glass waste canisters in a federal geologic repository. Total costs for processing of waste generated to the year 2000 are estimated to be about $2.9 billion (1984 dollars); incremental unit costs for DWPF and repository disposal activities range from $120,000 to $170,000 per canister depending on DWPF processing schedules. In a representative evaluation of process alternatives, the model is used to demonstrate cost effectiveness of adjustments in the frit content of the waste glass to reduce impacts of wastes generated by the salt decontamination operations. 13 references, 8 tables

Investigation of Various LiCl Waste Salt Purification Technologies

International Nuclear Information System (INIS)

Yung-Zun Cho; Hee-Chul Yang; Han-Soo Lee; In-Tae Kim

2008-01-01

Various purification research of LiCl waste molten salt generated from electroreduction process were tested. The purification of the LiCl waste salt very important in a various aspects, where the purification means separation of cesium and strontium form LiCl salt melts. In this study, for the separation of cesium and strontium from LiCl salt melts, precipitant agent addition techniques such as sulfate and carbonate addition method and, as a new attempt, zone freezing technique for concentration of cesium and strontium elements was investigated. As a results of this research, only strontium was carbonated by reaction with Li 2 CO 3 (cesium did not react with Li 2 CO 3 ). In case of sulfate addition method, both cesium and strontium were converted into their sulfate that is Cs 2 S 2 O 6 and SrSO 4 and maximum sulfate efficiency of cesium and strontium were about 72% and 95%, respectively. Cesium and strontium involved in LiCl molten salt could be concentrated in the molten salt by using zone freezing method. (authors)

Application of molten salt oxidation for the minimization and recovery of plutonium-238 contaminated wastes

International Nuclear Information System (INIS)

Wishau, R.; Ramsey, K.B.; Montoya, A.

1998-01-01

This paper presents the technical and economic feasibility of molten salt oxidation technology as a volume reduction and recovery process for 238 Pu contaminated waste. Combustible low-level waste material contaminated with 238 Pu residue is destroyed by oxidation in a 900 C molten salt reaction vessel. The combustible waste is destroyed creating carbon dioxide and steam and a small amount of ash and insoluble 2328 Pu in the spent salt. The valuable 238 Pu is recycled using aqueous recovery techniques. Experimental test results for this technology indicate a plutonium recovery efficiency of 99%. Molten salt oxidation stabilizes the waste converting it to a non-combustible waste. Thus installation and use of molten salt oxidation technology will substantially reduce the volume of 238 Pu contaminated waste. Cost-effectiveness evaluations of molten salt oxidation indicate a significant cost savings when compared to the present plans to package, or re-package, certify and transport these wastes to the Waste Isolation Pilot Plant for permanent disposal. Clear and distinct cost advantages exist for MSO when the monetary value of the recovered 238 Pu is considered

Deep geologic disposal of mixed waste in bedded salt: The Waste Isolation Pilot Plant

International Nuclear Information System (INIS)

Rempe, N.T.

1993-01-01

Mixed waste (i.e., waste that contains both chemically hazardous and radioactive components) poses a moral, political, and technical challenge to present and future generations. But an international consensus is emerging that harmful byproducts and residues can be permanently isolated from the biosphere in a safe and environmentally responsible manner by deep geologic disposal. To investigate and demonstrate such disposal for transuranic mixed waste, derived from defense-related activities, the US Department of Energy has prepared the Waste Isolation Pilot Plant (WIPP) near Carlsbad, New Mexico. This research and development facility was excavated approximately at the center of a 600 m thick sequence of salt (halite) beds, 655 m below the surface. Proof of the long-term tectonic and hydrological stability of the region is supplied by the fact that these salt beds have remained essentially undisturbed since they were deposited during the Late Permian age, approximately 225 million years ago. Plutonium-239, the main radioactive component of transuranic mixed waste, has a half-life of 24,500 years. Even ten half-lives of this isotope - amounting to about a quarter million years, the time during which its activity will decline to background level represent only 0.11 percent of the history of the repository medium. Therefore, deep geologic disposal of transuranic mixed waste in Permian bedded salt appears eminently feasible

[Adrenal tumours in childhood].

Science.gov (United States)

Martos-Moreno, G A; Pozo-Román, J; Argente, J

2013-09-01

This special article aims to summarise the current knowledge regarding the two groups of tumours with their origin in the adrenal gland: 1) adrenocortical tumours, derived from the cortex of the adrenal gland and 2) phaeochromocytomas and paragangliomas, neuroendocrine tumours derived from nodes of neural crest derived cells symmetrically distributed at both sides of the entire spine (paragangliomas [PG]). These PGs can be functioning tumors that secrete catecholamines, which confers their typical dark colour after staining with chromium salts (chromaffin tumors). Among these, the term phaeochromocytoma (PC) is restricted to those PGs derived from the chromaffin cells in the adrenal medulla (intra-adrenal PGs), whereas the term PG is used for those sympathetic or parasympathetic ones in an extra-adrenal location. We analyse the state of the art of their pathogenic and genetic bases, as well as their clinical signs and symptoms, the tests currently available for performing their diagnosis (biochemical, hormonal, imaging and molecular studies) and management (surgery, pre- and post-surgical medical treatment), considering the current and developing strategies in chemo- and radiotherapy. Copyright © 2013 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.

Engineering Options Assessment Report. Nitrate Salt Waste Stream Processing

Energy Technology Data Exchange (ETDEWEB)

Anast, Kurt Roy [Los Alamos National Lab. (LANL), Los Alamos, NM (United States)

2015-11-13

This report examines and assesses the available systems and facilities considered for carrying out remediation activities on remediated nitrate salt (RNS) and unremediated nitrate salt (UNS) waste containers at Los Alamos National Laboratory (LANL). The assessment includes a review of the waste streams consisting of 60 RNS, 29 above-ground UNS, and 79 candidate below-ground UNS containers that may need remediation. The waste stream characteristics were examined along with the proposed treatment options identified in the Options Assessment Report . Two primary approaches were identified in the five candidate treatment options discussed in the Options Assessment Report: zeolite blending and cementation. Systems that could be used at LANL were examined for housing processing operations to remediate the RNS and UNS containers and for their viability to provide repackaging support for remaining LANL legacy waste.

Engineering Options Assessment Report: Nitrate Salt Waste Stream Processing

Energy Technology Data Exchange (ETDEWEB)

Anast, Kurt Roy [Los Alamos National Lab. (LANL), Los Alamos, NM (United States)

2015-11-18

This report examines and assesses the available systems and facilities considered for carrying out remediation activities on remediated nitrate salt (RNS) and unremediated nitrate salt (UNS) waste containers at Los Alamos National Laboratory (LANL). The assessment includes a review of the waste streams consisting of 60 RNS, 29 aboveground UNS, and 79 candidate belowground UNS containers that may need remediation. The waste stream characteristics were examined along with the proposed treatment options identified in the Options Assessment Report . Two primary approaches were identified in the five candidate treatment options discussed in the Options Assessment Report: zeolite blending and cementation. Systems that could be used at LANL were examined for housing processing operations to remediate the RNS and UNS containers and for their viability to provide repackaging support for remaining LANL legacy waste.

Establishment of cooperation basis of joint research on the mixed waste molten salt oxidation technology

International Nuclear Information System (INIS)

Yang, Hee Chul; Cho, Y. J.; Kim, J. H.; Yoo, J. H.; Yun, H. C.; Lee, D. G.

2005-08-01

Molten salt oxidation, MSO for short, is a robust technology that can effectively treat mixed waste (radioactive waste including hazardous metals or organics). It can safely and economically treat the difficult wastes such as not-easily destroyable toxic organic waste, medical waste, chemical warfare and energetic materials such as propellant and explosives, all of which are not easily treated by an incinerator or other currently existing thermal treatment system. Therefore, molten salt oxidation technology should be developed and utilized to treat a lot of niche waste stored in the nuclear and environmental industries. So, if we put the MSO technology to practical use by Korea-Vietnam joint research, we can reduce R and D fund for MSO technology by ourselves and we can expect an export of the outcome of nuclear R and D in Korea. For Establishment of cooperation basis of joint research concerning molten salt oxidation technology between KOREA and VIETNAM, in this research, We invited two Vietnamese researchers and we introduced our experimental scale molten salt oxidation system in order to let them understand molten salt oxidation technology. We also visited Viet man and we consulted about molten salt oxidation process. We held seminar on the mixed waste molten salt oxidation technology, discussed on the joint research on the mixed waste molten salt oxidation technology and finally we wrote MOU for joint research

Establishment of cooperation basis of joint research on the mixed waste molten salt oxidation technology

Energy Technology Data Exchange (ETDEWEB)

Yang, Hee Chul; Cho, Y. J.; Kim, J. H.; Yoo, J. H.; Yun, H. C.; Lee, D. G

2005-08-01

Molten salt oxidation, MSO for short, is a robust technology that can effectively treat mixed waste (radioactive waste including hazardous metals or organics). It can safely and economically treat the difficult wastes such as not-easily destroyable toxic organic waste, medical waste, chemical warfare and energetic materials such as propellant and explosives, all of which are not easily treated by an incinerator or other currently existing thermal treatment system. Therefore, molten salt oxidation technology should be developed and utilized to treat a lot of niche waste stored in the nuclear and environmental industries. So, if we put the MSO technology to practical use by Korea-Vietnam joint research, we can reduce R and D fund for MSO technology by ourselves and we can expect an export of the outcome of nuclear R and D in Korea. For Establishment of cooperation basis of joint research concerning molten salt oxidation technology between KOREA and VIETNAM, in this research, We invited two Vietnamese researchers and we introduced our experimental scale molten salt oxidation system in order to let them understand molten salt oxidation technology. We also visited Viet man and we consulted about molten salt oxidation process. We held seminar on the mixed waste molten salt oxidation technology, discussed on the joint research on the mixed waste molten salt oxidation technology and finally we wrote MOU for joint research.

Radiolysis salt phenomenology: application to storage of high level radioactive waste

International Nuclear Information System (INIS)

Akram, Najib

1993-01-01

In France, rock salt is a candidate repository for highly radioactive waste. Rock salt contains water and adsorbed gases which can be released in boreholes after heating due to vitrified wastes. In addition, waste-induced irradiation in near-field conditions induce radiolytic reactions which also contribute to gas release. The aim of this work is to understand and evaluate the effects of heat and irradiation produced by waste containers in a deep disposal, primarily concerning gas production. This is justified by the impact of gases on long-term safety: toxicity, explosibility, chemical reactivity, pressure build-up. We have evidenced the influence of integrated dose, filling gases, temperature and grain size on an homogeneous medium (Asse Mine rock salt). We have then studied heterogeneous samples, which allowed to determine the influence of the chemical and mineralogical composition of rock salt (bedded rock salt from the Mine de Potasse d'Alsace). The role played by organic matter on gas production is important, leading for instance to high consumption rates of oxygen. Through this study, we have also considered the behaviour of clay-rich materials under irradiation. Our results constitute important bases for the future modelling of the phenomena which will take place in the near-field of a rock salt-type repository, especially concerning its long-term safety. (author) [fr

Neuropsychological assessment in prepubertal patients with congenital adrenal hyperplasia: preliminary study.

Science.gov (United States)

Somajni, F; Sovera, V; Albizzati, A; Russo, G; Peroni, P; Seragni, G; Lenti, C

2011-02-01

Individuals with congenital adrenal hyperplasia (CAH) provide a test population for the theory that elevated testosterone levels alter pre-/perinatal brain development. Seven prepuberal girls with CAH and seven matched controls has been submitted to a neuropsychological evaluation. We measured abilities where gender differences repeatedly has been observed or that had earlier shown differences between CAH subjects and controls. The following cognitive functions were tested: general intelligence, attention, verbal and non-verbal abilities, cerebral dominance for verbal and non-verbal material, frontal functions, peripheral dominance and motor fluency. Since several animal studies shown hippocampal morphological changes induced by prolonged hydrocortisone exposure, we also investigated memory functions. No differences were recorded between two groups on those abilities that are not sexually dimorphic. The mean general intelligence level of the patients was significantly lower than the controls', in agreement with previous studies. The verbal and non-verbal tasks revealed an age-related male-like pattern (i.e., verbal disadvantage) and an inversion of the hemispheric dominances. The latter observation was supported by a right-to-left shift of the peripheral dominances. The patients memory performances were all inferior to the controls'. The results are discussed in the light of possible hormonal influences. Our main findings support the hypothesis that elevated pre-/perinatal androgen exposure can influence some cognitive pattern of specific sexual dimorphic abilities in prepubertal subjects.

Neonatal Bartter Syndrome in association with congenital adrenal hyperplasia in a neonate - a rare combination.

Science.gov (United States)

Hussain, Shabbir

2016-05-01

Neonatal Bartter syndrome (NBS) is an autosomal recessive renal tubulopathy characterized by hypokalaemic, hypochloraemic metabolic alkalosis associated with increased urinary loss of sodium, potassium, calcium and chloride. There is hyperreninaemia and hyperaldosteronaemia but normotension. Congenital adrenal hyperplasia (CAH), another autosomal recessive condition, may present in the neonatal period with vomiting, hypovolaemia, failure to gain weight or ambiguous genitalia. We report a case of NBS and CAH combination in a neonate. A male neonate born at term was admitted with history of recurrent vomiting and dehydration episodes. Investigations revealed electrolytes imbalance, metabolic alkalosis, raised aldosterone and renin levels suggestive of NBS. He was treated successfully and discharged. He was re-admitted with the same symptoms. Further evaluation confirmed the presence of CAH as well. We report this case because of the rarity of this combination (NBS plus CAH) and to the best of our knowledge this is the first such case report from Pakistan.

Experimental results on salt concrete for barrier elements made of salt concrete in a repository for radioactive waste in a salt mine

International Nuclear Information System (INIS)

Gutsch, Alex-W.; Preuss, Juergen; Mauke, Ralf

2012-01-01

The Bartensleben rock salt mine in Germany was used as a repository for low and intermediate level radioactive waste from 1971 to 1991 and from 1994 to 1998. The repository with an overall volume of about 6 million m 3 has to be closed. Salt concrete is used for the refill of the voids of the repository. The concrete mixtures contain crushed salt instead of natural aggregates as the void filling material should be as similar to the salt rock as possible. Very high requirements regarding low heat development and little or even no cracking during concrete hardening had to be fulfilled even for the barrier elements made from salt concrete which separate the radioactive waste from the environment. Requirements for the salt concrete were set up with regard to the fluidity of the fresh concrete during the hardening process and its durability. In the view of a comprehensive numerical calculations of the temperature development and thermal stresses in the massive salt concrete elements of the backfill of the voids, experimental results for material properties of the salt concrete are presented: mixture of the salt concrete, thermodynamic properties (adiabatic heat release, thermal dilatation, thermal conductivity and heat capacity), mechanical short term properties, creep (under tension, under compression), autogenous shrinkage

Test plan for immobilization of salt-containing surrogate mixed wastes using polyester resins

International Nuclear Information System (INIS)

Biyani, R.K.; Douglas, J.C.; Hendrickson, D.W.

1997-01-01

Past operations at many Department of Energy (DOE) sites have resulted in the generation of several waste streams with high salt content. These wastes contain listed and characteristic hazardous constituents and are radioactive. The salts contained in the wastes are primarily chloride, sulfate, nitrate, metal oxides, and hydroxides. DOE has placed these types of wastes under the purview of the Mixed Waste Focus Area (MWFA). The MWFA has been tasked with developing and facilitating the implementation of technologies to treat these wastes in support of customer needs and requirements. The MWFA has developed a Technology Development Requirements Document (TDRD), which specifies performance requirements for technology owners and developers to use as a framework in developing effective waste treatment solutions. This project will demonstrate the use of polyester resins in encapsulating and solidifying DOE's mixed wastes containing salts, as an alternative to conventional and other emerging immobilization technologies

Test plan for immobilization of salt-containing surrogate mixed wastes using polyester resins

Energy Technology Data Exchange (ETDEWEB)

Biyani, R.K.; Douglas, J.C.; Hendrickson, D.W.

1997-07-07

Past operations at many Department of Energy (DOE) sites have resulted in the generation of several waste streams with high salt content. These wastes contain listed and characteristic hazardous constituents and are radioactive. The salts contained in the wastes are primarily chloride, sulfate, nitrate, metal oxides, and hydroxides. DOE has placed these types of wastes under the purview of the Mixed Waste Focus Area (MWFA). The MWFA has been tasked with developing and facilitating the implementation of technologies to treat these wastes in support of customer needs and requirements. The MWFA has developed a Technology Development Requirements Document (TDRD), which specifies performance requirements for technology owners and developers to use as a framework in developing effective waste treatment solutions. This project will demonstrate the use of polyester resins in encapsulating and solidifying DOE`s mixed wastes containing salts, as an alternative to conventional and other emerging immobilization technologies.

Waste isolation facility description: bedded salt

Energy Technology Data Exchange (ETDEWEB)

1976-09-01

The waste isolation facility is designed to receive and store three basic types of solidified wastes: high-level wastes, intermediate level high-gamma transuranic waste, and low-gamma transuranic wastes. The facility under consideration in this report is designed for bedded salt at a depth of approximately 1800 ft. The present design for the facility includes an area which would be used initially as a pilot facility to test the viability of the concept, and a larger facility which would constitute the final storage area. The total storage area in the pilot facility is planned to be 77 acres and in the fuel facility 1601 acres. Other areas for shaft operations and access would raise the overall size of the total facility to slightly less than 2,000 acres. The following subjects are discussed in detail: surface facilities, shaft design and characteristics, design and construction of the underground waste isolation facility, ventilation systems, and design requirements and criteria. (LK)

Waste isolation facility description: bedded salt

International Nuclear Information System (INIS)

1976-09-01

The waste isolation facility is designed to receive and store three basic types of solidified wastes: high-level wastes, intermediate level high-gamma transuranic waste, and low-gamma transuranic wastes. The facility under consideration in this report is designed for bedded salt at a depth of approximately 1800 ft. The present design for the facility includes an area which would be used initially as a pilot facility to test the viability of the concept, and a larger facility which would constitute the final storage area. The total storage area in the pilot facility is planned to be 77 acres and in the fuel facility 1601 acres. Other areas for shaft operations and access would raise the overall size of the total facility to slightly less than 2,000 acres. The following subjects are discussed in detail: surface facilities, shaft design and characteristics, design and construction of the underground waste isolation facility, ventilation systems, and design requirements and criteria

Different Methods for Conditioning Chloride Salt Wastes

International Nuclear Information System (INIS)

De Angelis, G.; Fedeli, C.; Capone, M.; Marzo, G.A.; Mariani, M.; Da Ros, M.; Giacobbo, F.; Macerata, E.; Giola, M.

2015-01-01

Three different methods have been used to condition chloride salt wastes coming from pyro-processes. Two of them allow to synthesise sodalite, a naturally occurring mineral containing chlorine: the former, starting from Zeolite 4A, which transforms the zeolite into sodalite; the latter, which starts from kaolinite, giving sodalite as well. In addition, a new matrix, termed SAP (SiO 2 -Al 2 O 3 -P 2 O 5 ), has been synthesised. It is able to form different mineral phases which occlude fission metals. The products from the different processes have been fully characterised. In particular the chemical durability of the final waste forms has been determined using the standard product consistency test. According to the results obtained, SAP seems to be a promising matrix for the incorporation of chloride salt wastes from pyro-processes. Financial support from the Nuclear Fission Safety Programme of the European Union (projects ACSEPT, contract FP7-CP-2007- 211 267, and SACSESS, Collaborative Project 323282), as well as from Italian Ministry for Economic Development (Accordo di Programma: Piano Annuale di Realizzazione 2008-2009) is gratefully acknowledged. (authors)

Concepts and Technologies for Radioactive Waste Disposal in Rock Salt

Directory of Open Access Journals (Sweden)

Wernt Brewitz

2007-01-01

Full Text Available In Germany, rock salt was selected to host a repository for radioactive waste because of its excellent mechanical properties. During 12 years of practical disposal operation in the Asse mine and 25 years of disposal in the disused former salt mine Morsleben, it was demonstrated that low-level wastes (LLW and intermediate-level wastes (ILW can be safely handled and economically disposed of in salt repositories without a great technical effort. LLW drums were stacked in old mining chambers by loading vehicles or emplaced by means of the dumping technique. Generally, the remaining voids were backfilled by crushed salt or brown coal filter ash. ILW were lowered into inaccessible chambers through a borehole from a loading station above using a remote control.Additionally, an in-situ solidification of liquid LLW was applied in the Morsleben mine. Concepts and techniques for the disposal of heat generating high-level waste (HLW are advanced as well. The feasibility of both borehole and drift disposal concepts have been proved by about 30 years of testing in the Asse mine. Since 1980s, several full-scale in-situ tests were conducted for simulating the borehole emplacement of vitrified HLW canisters and the drift emplacement of spent fuel in Pollux casks. Since 1979, the Gorleben salt dome has been investigated to prove its suitability to host the national final repository for all types of radioactive waste. The “Concept Repository Gorleben” disposal concepts and techniques for LLW and ILW are widely based on the successful test operations performed at Asse. Full-scale experiments including the development and testing of adequate transport and emplacement systems for HLW, however, are still pending. General discussions on the retrievability and the reversibility are going on.

Pyrolytic conversion of plastic and rubber waste to hydrocarbons with basic salt catalysts

Science.gov (United States)

Wingfield, Jr., Robert C.; Braslaw, Jacob; Gealer, Roy L.

1985-01-01

The invention relates to a process for improving the pyrolytic conversion of waste selected from rubber and plastic to low molecular weight olefinic materials by employing basis salt catalysts in the waste mixture. The salts comprise alkali or alkaline earth compounds, particularly sodium carbonate, in an amount of greater than about 1 weight percent based on the waste feed.

Potential for creation of a salt dome following disposal of radioactive waste in a salt layer

International Nuclear Information System (INIS)

Fries, G.

1987-01-01

The study aims at quantifying the possibility of creation of a salt dome from a salt layer in which heat-emitting radioactive waste would be buried. Volume 1 describes the results of numerical computer simulations, and of laboratory-scale models in centrifuges. Volume 2 envisages, in a geological perspective, the origin of salt domes, the mechanisms of thei formation, and the associated parameters [fr

Potential for creation of a salt dome following disposal of radioactive waste in a salt layer

International Nuclear Information System (INIS)

Charo, L.; Habib, P.

1987-01-01

The study aims at quantifying the possibility of creation of a salt dome from a salt layer in which heat-emitting radioactive waste would be buried. Volume 1 describes the results of numerical computer simulations, and of laboratory-scale models in centrifuges. Volume 2 envisages, in a geological perspective, the origin of salt domes, the mechanisms of their formation, and the associated parameters [fr

Waste salt disposal at the Savannah River Plant

International Nuclear Information System (INIS)

Langton, C.A.; Oblath, S.B.; Pepper, D.W.; Wilhite, E.L.

1986-01-01

Waste salt solution, produced during processing of high-level nuclear waste, will be incorporated in a cement matrix for emplacement in an engineered disposal facility. Wasteform characteristics and disposal facility details will be presented along with results of a field test of wasteform contaminant release and of modeling studies to predict releases. 5 refs., 11 figs., 5 tabs

Waste package for a repository located in salt

International Nuclear Information System (INIS)

Basham, S.J. Jr.

1983-01-01

This paper describes the current status of the waste package designs for salt repositories. The status of the supporting studies of environment definition, corrosion of containment materials, and leaching of waste forms is also presented. Emphasis is on the results obtained in FY 83 and the planned effort in FY 84. 8 references, 3 figures, 1 table

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

Directory of Open Access Journals (Sweden)

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

Treatment of waste salt from the advanced spent fuel conditioning process (II) : optimum immobilization condition

International Nuclear Information System (INIS)

Kim, Jeong Guk; Lee, Jae Hee; Yoo, Jae Hyung; Kim, Joon Hyung

2004-01-01

Since zeolite is known to be stable at a high temperature, it has been reported as a promising immobilization matrix for waste salt. The crystal structure of dehydrated zeolite A breaks down above 1060 K, resulting in the formation of an amorphous solid and re-crystallization to beta-Cristobalite. This structural degradation depends on the existence of chlorides. When contacted to HCl, zeolite 4A is not stable even at 473 K. The optimum consolidation condition for LiCl salt waste from the oxide fuel reduction process based on the electrochemical method (Advanced spent fuel Conditioning Process; ACP) has been studied using zeolite A since 2001. Actually the constituents of waste salt are water-soluble. And, alkali halides are known to be readily radiolyzed to yield interstitial halogens and metal colloids. For disposal in a geological repository, the waste salt must meet the acceptance criteria. For a waste form containing chloride salt, two of the more important criteria are leach resistance and waste form durability. In this work, we prepared some samples with different mixing ratios of LiCl salt to zeolite A, and then compared some characteristics such as thermal stability, salt occlusion, free chloride content, leach resistance, mixing effect, etc

Immobilization of IFR salt wastes in mortar

International Nuclear Information System (INIS)

Fisher, D.F.; Johnson, T.R.

1988-01-01

Portland cement-base mortars are being considered for immobilizing chloride salt wastes from the fuel cycle of an integral fast reactor (IFR). The IFR is a sodium-cooled fast reactor with metal fuel. It has a close-coupled fuel cycle in which fission products are separated from the actinides in an electrochemical cell operating at 500 degrees C. This cell has a cadmium anode and a liquid salt electrolyte. The salt will be a low-melting mixture of alkaline and alkaline earth chlorides. This paper discusses one method being considered for immobilizing this treated salt, to disperse it in a portland cement-base motar, which would then be sealed in corrosion-resistant containers. For this application, the grout must be sufficiently fluid that it can be pumped into canisters where it will solidify into a strong, leach-resistant material

Cerebral salt wasting following tuberculous meningoencephalitis in an infant

Directory of Open Access Journals (Sweden)

Syed Ahmed Zaki

2012-01-01

Full Text Available In patients with central nervous system disease, life-threatening hyponatremia can result from either the syndrome of inappropriate secretion of antidiuretic hormone or cerebral salt wasting. Clinical manifestations of the two conditions may be similar, but their pathogeneses and management protocols are different. Cerebral salt wasting syndrome is a disorder in which excessive natriuresis and hyponatremia occurs in patients with intracranial diseases. We report a 6-month-old girl with CSWS associated with tuberculous meningoencephalitis. She was diagnosed as having CSWS on the basis of hypovolemia, polyuria, natriuresis, and the relatively high level of fractional excretion of uric acid. Aggressive replacement of urine salt and water losses using 0.9% or 3% sodium chloride was done. Fludrocortisone was started at 0.1 mg twice daily on the seventh day of admission and was continued for 17 days.

Volume reduction of waste contaminated by fission product elements and plutonium using molten salt combustion

International Nuclear Information System (INIS)

McKenzie, D.E.; Grantham, L.F.; Paulson, R.B.

1979-01-01

In the Molten Salt Combustion Process, transuranic or β-γ organic waste and air are continuously introduced beneath the surface of a sodium carbonate-containing melt at a temperature of about 800 0 C. Complete combustion of the organic material to carbon dioxide and steam occurs without the conversion of nitrogen to nitrogen oxides. The noxious gases formed by combustion of the chloride, sulfur or phosphorus content of the waste instantly react with the melt to form the corresponding sodium compounds. These compounds as well as the ash and radionuclides are retained in the molten salt. The spent salt is either fused cast into an engineered disposal container or processed to recover salt and plutonium. Molten salt combustion reduces the waste to about 2% of its original volume. Many reactor or reprocessing wastes which cannot be incinerated without difficulty are readily combusted in the molten salt. A 50 kg/hr molten salt combustion system is being designed for the Radioactive Waste Management Complex at the Idaho National Engineering Laboratory. Construction of the combustor started during 1977, and combustor startup was scheduled for the spring of 1978

Destruction of high explosives and wastes containing high explosives using the molten salt destruction process

International Nuclear Information System (INIS)

Upadhye, R.S.; Brummond, W.A.; Pruneda, C.O.

1992-01-01

This paper reports the Molten Salt Destruction (MSD) Process which has been demonstrated for the destruction of HE and HE-containing wastes. MSD has been used by Rockwell International and by Anti-Pollution Systems to destroy hazardous wastes. MSD converts the organic constituents (including the HE) of the waste into non-hazardous substances such as carbon dioxide, nitrogen and water. In the case of HE-containing mixed wastes, any actinides in the waste are retained in the molten salt, thus converting the mixed wastes into low-level wastes. (Even though the MSD process is applicable to mixed wastes, this paper will emphasize HE-treatment.) The destruction of HE is accomplished by introducing it, together with oxidant gases, into a crucible containing a molten salt, such as sodium carbonate, or a suitable mixture of the carbonates of sodium, potassium, lithium and calcium. The temperature of the molten salt can be between 400 to 900 degrees C. The combustible organic components of the waste react with oxygen to produce carbon dioxide, nitrogen and steam

Salt-occluded zeolite waste forms: Crystal structures and transformability

International Nuclear Information System (INIS)

Richardson, J.W. Jr.

1996-01-01

Neutron diffraction studies of salt-occluded zeolite and zeolite/glass composite samples, simulating nuclear waste forms loaded with fission products, have revealed complex structures, with cations assuming the dual roles of charge compensation and occlusion (cluster formation). These clusters roughly fill the 6--8 angstrom diameter pores of the zeolites. Samples are prepared by equilibrating zeolite-A with complex molten Li, K, Cs, Sr, Ba, Y chloride salts, with compositions representative of anticipated waste systems. Samples prepared using zeolite 4A (which contains exclusively sodium cations) as starting material are observed to transform to sodalite, a denser aluminosilicate framework structure, while those prepared using zeolite 5A (sodium and calcium ions) more readily retain the zeolite-A structure. Because the sodalite framework pores are much smaller than those of zeolite-A, clusters are smaller and more rigorously confined, with a correspondingly lower capacity for waste containment. Details of the sodalite structures resulting from transformation of zeolite-A depend upon the precise composition of the original mixture. The enhanced resistance of salt-occluded zeolites prepared from zeolite 5A to sodalite transformation is thought to be related to differences in the complex chloride clusters present in these zeolite mixtures. Data relating processing conditions to resulting zeolite composition and structure can be used in the selection of processing parameters which lead to optimal waste forms

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.

Fetal adrenal gland enlargement - prenatal and postnatal management.

Science.gov (United States)

Lackova, Eliska; Cunderlik, Anton; Ticha, Lubica; Gabor, Maria

2017-11-01

The enlargement of suprarenal gland is related to preterm birth and the birth weight. The ultrasound measurement of fetal adrenal gland volume may identify women at risk for impending preterm birth. The aim of our study was to investigate the newborns in the region of western Slovakia followed up due to suprarenal gland enlargement. To set the ratio of prenatally diagnosed suprarenal gland enlargment, postnatal managment and treatment and interventions. The newborns with congenital adrenal hyperplasia were excluded. We have analyzed 6 years of medical records of all cases from the western Slovakia region of suprarenal gland enlargement encountered to 1st Pediatric Department, Children's University Hospital Bratislava Republic in the time period of January 2010 to Janurary 2016. The diagnosis of suprarenal gland enlargement was set by ultrasound examination performed on the 4th postnatal day as an overall screening test. Newborns with positive laboratory screening on congenital adrenal hyperplasia (CAH) were excluded from our study. We analyzed the origin of surarenal gland enlargement, gestation week on the due date, the birth weight and other comorbidities and genetic pathologies in newborns with the enlarged suprarenal glands. There were 6 newborns followed up due to suprarenal gland enlargement. All of the patients had diagnosed the adrenal haemorrhage. Adrenal lesions like adrenal cysts or neuroblastomas were not confirmed. All of the adrenal enlargements were benign with no need of other medical or surgical intervention. None of the newborn patients had other genetic abnormalities, mineral or hormonal imbalances, problems with arterial pressure or haemodynamic instability. All of the patients underwent at least 5 prenatal ultrasound tests and at least 2 postnatal ultrasound measurements. The avarage birth weight was 3030 grams (2700 grams - to 3750 grams). The avarage birth lenght was 50 cm (47 centimeter to 53 cm).The average gestation week (gw) on due date

Nuclear waste in sea or salt? No, wrong

International Nuclear Information System (INIS)

Damveld, H.; Van Duin, S.; Bannink, D.

1994-04-01

Eighteen years of successful action against ocean dumping and storage of nuclear waste in salt domes are reviewed for the Dutch situation. The aim of this book is to hand some support to those who want to act against trial borings, in particular the people living close to the most important salt domes in the Netherlands: Ternaard, Zuidwending, Pieterburen, Onstwedde, Winschoten, Schoonlo and Gasselte-Drouwen. In 1976 the Interdepartmental Commission on Nuclear Energy with its subcommission Radioactive Substances (ICK-RAS) was installed, along with a number of working groups, responsible for research. From 1978 onwards ocean dumping operations were accompanied by blockades and legal procedures, which led to a situation of the last dumping in 1982. The Dutch government then focused on nuclear waste storage in salt domes for which the OPLA research program was started. OPLA is the Dutch abbreviation for Storage on Land. The final report (phase 1 and 1a) of OPLA was published on 15 October 1993 as annex to the Dossier Nuclear Energy of the Dutch government. It has been decided that phase 1a is not followed by trial drillings, as planned before. Some critical remarks are made regarding the rounds of public participation and the notion of permanent retrievability of stored nuclear waste. Extensive use has been made of documentation from the Dutch government and parliament, and other literature and information sources

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

Directory of Open Access Journals (Sweden)

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

Temperature distributions in a salt formation used for the ultimate disposal of high-level radioactive waste

International Nuclear Information System (INIS)

Ploumen, P.

1980-01-01

In the Federal Republic of Germany the works on waste disposal is focussed on the utilization of a salt formation for ultimate disposal of radioactive wastes. Heat released from the high-level waste will be dissipated in the salt and the surrounding geologic formations. The occuring temperature distributions will be calculated with computer codes. A survey of the developed computer codes will be shown; the results for a selected example, taking into account the loading sequence of the waste, the mine ventilation as well as an air gap between the waste and the salt, will be discussed. Furthermore it will be shown that by varying the disposal parameters, the maximum salt temperature can be below any described value. (Auth.)

Association of leptin with cardiometabolic factors in schoolchildren and adolescents with congenital adrenal hyperplasia.

Science.gov (United States)

Zurita-Cruz, Jessie Nallely; Villasís-Keever, Miguel Ángel; Damasio-Santana, Leticia; Manuel-Apolinar, Leticia; Ferrusca-Ceja, Rosalba; Nishimura-Meguro, Elisa; Rivera-Hernández, Aleida de J; Garrido-Magaña, Eulalia

2018-01-01

In congenital adrenal hyperplasia (CAH), obesity, hyperinsulinemia and leptin levels are increased. To identify the frequency of cardiometabolic risk factors (CRF) in children and adolescents with CAH and to explore the relationship with leptin levels. Cross-sectional study of 40 patients who underwent anthropometric measurements and had fasting glucose, insulin, triglycerides, 17-hidroxyprogesterone, leptin, HDL and LDL-cholesterol assessed. The patients were classified according to the number of CRFs, and leptin levels were analyzed with the Kruskal-Wallis test. Pearson's correlation was applied between leptin, body mass index (BMI) z-score and body fat percentage. Fifty percent of the patients had obesity and overweight, 59% had hypertriglyceridemia, 40%, hypoalphalipoproteinemia, 27.5%, high LDL-cholesterol and 22.5% insulin resistance. There was positive correlation between leptin and body fat percentage (r = 0.64), BMI z-score (r = 0.55) and the number of CRFs (r = 0.65). In the obesity-adjusted multivariate analysis, leptin levels were associated with the number of CRFs. CAH had a high frequency of CRFs and leptin appeared to be associated with a more adverse cardiometabolic profile in subjects with obesity and overweight. Copyright: © 2018 SecretarÍa de Salud.

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.

Application of molten salt oxidation for the minimization and recovery of plutonium-238 contaminated wastes

International Nuclear Information System (INIS)

Wishau, R.

1998-05-01

Molten salt oxidation (MSO) is proposed as a 238 Pu waste treatment technology that should be developed for volume reduction and recovery of 238 Pu and as an alternative to the transport and permanent disposal of 238 Pu waste to the WIPP repository. In MSO technology, molten sodium carbonate salt at 800--900 C in a reaction vessel acts as a reaction media for wastes. The waste material is destroyed when injected into the molten salt, creating harmless carbon dioxide and steam and a small amount of ash in the spent salt. The spent salt can be treated using aqueous separation methods to reuse the salt and to recover 99.9% of the precious 238 Pu that was in the waste. Tests of MSO technology have shown that the volume of combustible TRU waste can be reduced by a factor of at least twenty. Using this factor the present inventory of 574 TRU drums of 238 Pu contaminated wastes is reduced to 30 drums. Further 238 Pu waste costs of $22 million are avoided from not having to repackage 312 of the 574 drums to a drum total of more than 4,600 drums. MSO combined with aqueous processing of salts will recover approximately 1.7 kilograms of precious 238 Pu valued at 4 million dollars (at $2,500/gram). Thus, installation and use of MSO technology at LANL will result in significant cost savings compared to present plans to transport and dispose 238 Pu TRU waste to the WIPP site. Using a total net present value cost for the MSO project as $4.09 million over a five-year lifetime, the project can pay for itself after either recovery of 1.6 kg of Pu or through volume reduction of 818 drums or a combination of the two. These savings show a positive return on investment

Radiological consequences associated with human intrusion into radioactive waste repositories in salt formations

International Nuclear Information System (INIS)

Jacquier, P.

1989-01-01

The assessment of the radiological impact of human intrusion scenarios is extremely important in the case of repositories located in salt formations, since salt is obviously a valuable economic resource. Salt formations also represent a suitable medium for mining storage caverns for oil and gas. The scenario considered in this report is that of solution mining in salt formations to produce salt for human consumption. It is postulated that the salt is extracted by excavating a cavern through solution-mining and that, in the course of cavern enlargement, the waste is intercepted and drops to the bottom of the cavern. We have assumed that the intrusion takes place 500 or even 2 500 years after the repository has been sealed. The cases considered involve high-level vitrified waste or cemented alpha waste. The paper describes the assumptions on which the scenario is based and uses a simplified model to assess the radiological consequences associated with the ingestion of contaminated salt. The paper also provides details of a sensitivity/uncertainty analysis which identified several areas in which experimental studies should be either initiated or continued [fr

Development and characterization of new high-level waste form containing LiCl KCl eutectic salts for achieving waste minimization from pyroprocessing

International Nuclear Information System (INIS)

Cho, Yong Zun; Kim, In Tae; Park, Hwan Seo; Ahn, Byeung Gil; Eun, Hee Chul; Son, Seock Mo; Ah, Su Na

2011-12-01

The purpose of this project is to develop new high level waste (HLW) forms and fabrication processes to dispose of active metal fission products that are removed from electrorefiner salts in the pyroprocessing based fuel cycle. The current technology for disposing of active metal fission products in pyroprocessing involves non selectively discarding of fission product loaded salt in a glass-bonded sodalite ceramic waste form. Selective removal of fission products from the molten salt would greatly minimize the amount of HLW generated and methods were developed to achieve selective separation of fission products during a previous I NERI research project (I NERI 2006 002 K). This I NERI project proceeds from the previous project with the development of suitable waste forms to immobilize the separated fission products. The Korea Atomic Energy Research Institute (KAERI) has focused primarily on developing these waste forms using surrogate waste materials, while the Idaho National Laboratory (INL) has demonstrated fabrication of these waste forms using radioactive electrorefiner salts in hot cell facilities available at INL. Testing and characterization of these radioactive materials was also performed to determine the physical, chemical, and durability properties of the waste forms

Technical support for GEIS: radioactive waste isolation in geologic formations. Volume 16. Repository preconceptual design studies: BPNL waste forms in salt

International Nuclear Information System (INIS)

1978-04-01

This volume, Volume 16, ''Repository Preconceptual Design Studies: BPNL Waste Forms in Salt,'' is one of a 23 volume series, ''Technical Support for GEIS: Radioactive Waste Isolation in Geologic Formations,'' Y/OWI/TM-36, which supplements the ''Contribution to Draft Generic Environmental Impact Statement on Commercial Waste Management: Radioactive Waste Isolation in Geologic Formations,'' Y/OWI/TM-44. The series provide a more complete technical basis for the preconceptual designs, resource requirements, and environmental source terms associated with isolating commercial LWR wastes in underground repositories in salt, granite, shale and basalt. Wastes are considered from three fuel cycles: uranium and plutonium recycling, no recycling of spent fuel and uranium-only recycling. This document describes a preconceptual design for a nuclear waste storage facility in salt. The waste forms assumed to arrive at the repository were supplied by Battelle Pacific Northwest Laboratories (BPNL). The facility design consists of several chambers excavated deep within a geologic formation together with access shafts and supportive surface structures. The facility design provides for: receiving and unloading waste containers; lowering them down shafts to the mine level; transporting them to the proper storage area and emplacing them in mined storage rooms. Drawings of the facility design are contained in TM-36/17, ''Drawings for Repository Preconceptual Design Studies: BPNL Waste Forms in Salt.''

Modeling of waste/near field interactions for a waste repository in bedded salt: the Dynamic Network (DNET) model

International Nuclear Information System (INIS)

Cranwell, R.M.

1983-01-01

The Fuel Cycle Risk Analysis Division of Sandia National Laboratories has been funded by the US Nuclear Regulatory Commission to develop a methodology for use in assessing the long-term risk from the disposal of radioactive wastes in deep geologic formations. As part of this program, the Dynamic Network (DNET) model was developed to investigate waste/near field interactions associated with the disposal of radioactive wastes in bedded salt formations. The model is a quasi-multi-dimensional network model with capabilities for simulating processes such as fluid flow, heat transport, salt dissolution, salt creep, and the effects of thermal expansion and subsedence on the rock units surrounding the repository. The use of DNET has been demonstrated in the analysis of a hypothetical disposal site containing a bedded salt formation as the host medium for the repository. An example of this demonstration analysis is discussed. Furthermore, the outcome of sensitivity analyses performed on the DNET model are presented

Interim performance specifications for conceptual waste-package designs for geologic isolation in salt repositories

International Nuclear Information System (INIS)

1983-06-01

The interim performance specifications and data requirements presented apply to conceptual waste package designs for all waste forms which will be isolated in salt geologic repositories. The waste package performance specifications and data requirements respond to the waste package performance criteria. Subject areas treated include: containment and controlled release, operational period safety, criticality control, identification, and waste package performance testing requirements. This document was generated for use in the development of conceptual waste package designs in salt. It will be revised as additional data, analyses, and regulatory requirements become available

X-ray diffraction of slag-based sodium salt waste forms

Energy Technology Data Exchange (ETDEWEB)

Langton, C. A. [Savannah River Site (SRS), Aiken, SC (United States). Savannah River National Lab. (SRNL); Missimer, D. M. [Savannah River Site (SRS), Aiken, SC (United States). Savannah River National Lab. (SRNL)

2014-09-30

The attached report documents sample preparation and x-ray diffraction results for a series of cement and blended cement matrices prepared with either water or a 4.4 M Na salt solution. The objective of the study was to provide initial phase characterization for the Cementitious Barriers Partnership reference case cementitious salt waste form. This information can be used to: 1) generate a base line for the evolution of the waste form as a function of time and conditions, 2) potentially to design new binders based on mineralogy of the binder, 3) understand and predict anion and cation leaching behavior of contaminants of concern, and 4) predict performance of the waste forms for which phase solubility and thermodynamic data are available.

Addison disease’s in puppy, congenital adrenal hyperplasia (adrenal 21 alpha hydroxilase deficiency) and Hypoparathyroidism in Dogs

OpenAIRE

Castillo, Víctor; Ortemberg, L.

2009-01-01

La enfermedad de Addison (EA), la hiperplasia adrenal congénita (HAC) por déficit de 21 αhidroxilasa adrenal y el hipoparatiroidismo (HoPTH) son de diagnóstico poco frecuente, representando el 3% (EA) y 0,5% (tanto HAC como HoPTH) de las enfermedades endócrinas atendidas. Se describen 1 caso de EA congénito (collie de 6 meses de edad), 2 casos de HAC (una caniche toy y una labradora, ambas de 2 años) y 2 de HoPTH (cocker spaniel de 11 meses y una mestiza de 7 años). Por la signología clínica ...

Alternative Electrochemical Salt Waste Forms, Summary of FY11-FY12 Results

Energy Technology Data Exchange (ETDEWEB)

Riley, Brian J. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Mccloy, John S. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Crum, Jarrod V. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Lepry, William C. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Rodriguez, Carmen P. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Windisch, Charles F. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Matyas, Josef [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Westman, Matthew P. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Rieck, Bennett T. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Lang, Jesse B. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Olszta, Matthew J. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Pierce, David A. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States)

2014-01-17

The Fuel Cycle Research and Development Program, sponsored by the U.S. Department of Energy Office of Nuclear Energy, is currently investigating alternative waste forms for wastes generated from nuclear fuel processing. One such waste results from an electrochemical separations process, called the “Echem” process. The Echem process utilizes a molten KCl-LiCl salt to dissolve the fuel. This process results in a spent salt containing alkali, alkaline earth, lanthanide halides and small quantities of actinide halides, where the primary halide is chloride with a minor iodide fraction. Pacific Northwest National Laboratory (PNNL) is concurrently investigating two candidate waste forms for the Echem spent-salt: high-halide minerals (i.e., sodalite and cancrinite) and tellurite (TeO2)-based glasses. Both of these candidates showed promise in fiscal year (FY) 2009 and FY2010 with a simplified nonradioactive simulant of the Echem waste. Further testing was performed on these waste forms in FY2011 and FY2012 to assess the possibility of their use in a sustainable fuel cycle. This report summarizes the combined results from FY2011 and FY2012 efforts.

Chemical modeling of nuclear waste repositories in the salt repository project

International Nuclear Information System (INIS)

Jansen, G.; Raines, G.E.; Kircher, J.F.; Hubbard, N.

1985-01-01

Salt deposits contain small amounts of water as brine in fluid inclusions in halite and in hydrous minerals, e.g., clays, kieserite (MgSO 4 . H 2 O) and carnallite (KMgCl 3 . 6H 2 O). For the candidate salt deposits, the total amounts of water as volume % brine are: Palo Duro Basin, Texas, approximately 1.8; Paradox Basin, Utah, approximately 5.0 for the carnallite-marker zone, and less than approximately 0.5 below this zone; Gulf Coast salt domes, less than 0.15. For the Palo Duro and Paradox salt, the brines are Mg-rich (approximately 20,000 mg/L to approximately 100,000 mg/L) and sometimes Ca-rich (up to about 20,000 mg/L) NaCl brines. Brine migration calculations have been made using calculations of the time-variant thermal gradient around the waste packages and conservatively high brine volumes in the salt (5.0 volume % for the Texas and Utah sites and 0.5 volume % for the Gulf Coast) as input data. The maximum amounts of brine that eventually migrate to each waste package are about 1.0m 3 (for 5.0 volume % brine) and 0.2m 3 (for 0.5 volume % brine). With current conceptual designs for waste package overpacks (10 to 15 cm thick low-carbon steel), the waste package is not breached by uniform corrosion within 10,000 years. In brines this material thus far shows only uniform corrosion. For the expected conditions, where the brine is provided solely by brine migration, the brine is consumed by reaction with the iron of the overpack nearly as fast as it migrates to the waste package. Therefore, for the expected conditions, data about corrosion rates, radiolysis, etc., are not important. However, it is essential that accurate volumes of in-migrating brine can be calculated

Trial storage of high-level waste in the Asse II salt mine

International Nuclear Information System (INIS)

1984-01-01

This report covers a second phase of the work performed by GSF and KfK in the Asse II salt mine, with a view to disposal of radioactive waste in salt formations. New items of the research were geophysical investigations of the behaviour of heated salt and preparation of a trial storage in the Asse II salt mine

Study on application of molten salt oxidation technology (MSO) for PVC wastes treatment

International Nuclear Information System (INIS)

Tran Thu Ha; Nguyen Hong Quy; Pham Quoc Ky; Nguyen Quang Long; Vuong Thu Bac; Dang Duc Nhan

2007-01-01

The project 'Study on application of molten salt oxidation (MSO) for PVC plastic wastes treatment' aims at three followings: 1) Installation of lab-scale MSO unit with essential compositions builds up foundation for the 2) estimation of waste destruction efficiency of the technology. 3) Based on the results of testing PVC - the chlorinated organic wastes on the lab-scale unit, the ability of the technology application at pilot-scale level will be primary estimated. The adjustment and correction of some compositions in the lab-scale unit theoretically designed during experiment overcame the shortages by design and fabrication such as heat distribution regime, feeding wastes and draining spent salt. These solutions adapt to the technical requirement of operation as well as scientific requirement of the research on MSO process. PVC waste treatment was tested on the MSO lab-scale unit in different conditions of operation temperature, superficial air velocity related to air/oxygen feeding rate, waste feeding rate. The testing results showed that destruction efficiency of chlorine in MSO technology was almost absolute. HCl and Cl 2 emission were insignificant in different operation conditions. HCl and Cl 2 emission depend on resident time and nature of molten salt. However, with inherent attributes of MSO technology emission of CO is not avoided in processing waste treatment. Therefore, finding active solutions for reduction CO emission is essential to complete the technology. The experiments also were carried in conditions of single molten salt (Na 2 CO 3 ) and molten (Na 2 CO 3 - K 2 CO 3 ) eutectic. The comparison of efficiency of these tests gives idea of using molten salt eutectic to reduce operation cost in MSO technology. Based on operation parameters and scientific verification results during experiments, the introductory procedure of waste treatment by MSO process was built up. Thereby, primary estimation of development of the technology in pilot-scale is given

Some geotechnical problems related to underground waste disposal in salt formations

International Nuclear Information System (INIS)

Berest, P.

1993-01-01

Nuclear waste disposal in deep salt formations is an option considered by several countries. Rock salt is a very impervious medium, but can be easily leached; selection of an appropriate disposal formation must account for natural protections of the formation as regards water movements. It must be checked that such initially favourable characteristics will not be affected by the existence of shafts and galleries, or by the important heat output generated by vitrified wastes. The discussion is uneasy, for a comprehensive rheological model for rock salt is difficult to set and to be extrapolated to large time scales; some methodological problems are raised by use of numerical computations. (author). 22 refs., 2 figs

The thermo-mechanical behaviour of a salt dome with a heat-generating waste repository

International Nuclear Information System (INIS)

Janssen, L.G.J.; Prij, J.; Kevenaar, J.W.A.M.; Jong, C.J.T.; Klok, J.; Beemsterboer, C.

1984-01-01

This report reviews the analytical work on the disposal of radioactive waste in salt domes performed at ECN in the period 1 January 1980 to 31 December 1982. Chapter 4 in the main report covers the global temperature and deformation analyses of the salt dome and the surrounding rocks. The attached three topical reports cover self-contained parts of the study. The computer program TASTE developed to analyse, at acceptable cost and with, for engineering purposes, sufficient accuracies, the temperature rises in the salt dome due to the stored heat-generating waste is described in Annex 1. Annex 2 gives a description of the extended finite element program GOLIA. The program has been extended to make it suitable for the creep analysis of salt domes with repositories of heat-generating waste. The study on the closing and sealing of boreholes wit heat-generating waste is reported in Annex 3

Backfill barriers for nuclear waste repositories in salt

Energy Technology Data Exchange (ETDEWEB)

Nowak, E J; Odoj, R; Merz, E [eds.

1981-06-01

Backfill materials were evaluated for containment of radionuclides, chemical modification of brine, and sensitivity to hydrothermal conditions. Experimental conditions were relevant to nuclear waste isolation in bedded salt. They were based on geologic conditions at the site of the Waste Isolation Pilot Plant (WIPP) in southeastern New Mexico, USA. Conclusions are: backfill mixtures surrounding the waste form and canister can provide a neutral or slightly acidic, potentially reducing environment, prevent convective aqueous flow, and act as an effective radionuclide migration barrier; bentonite is likely to remain hydrothermally stable but potentially sensitive to waste package interactions which could alter the pH, the ratio of dissolved ions, or the sorption properties of radionuclide species; effects of irradiation from high level waste should be investigated.

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

OpenAIRE

Yaser Ramírez Benítez; Hussimy Marchena Morera

2008-01-01

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 n...

An improvement study on the closed chamber distillation system for recovery of renewable salts from salt wastes containing radioactive rare earth compounds

International Nuclear Information System (INIS)

Eun, H.C.; Cho, Y.Z.; Lee, T.K.; Kim, I.T.; Park, G.I.; Lee, H.S.

2013-01-01

In this paper, an improvement study on the closed chamber distillation system for recovery of renewable salts from salt wastes containing radioactive rare earth compounds was performed to determine optimum operating conditions. It was very important to maintain the pressure in the distillation chamber below 10 Torr for a high efficiency (salt recovery >99 %) of the salt distillation. This required increasing the salt vaporization and condensation rates in the distillation system. It was confirmed that vaporization and condensation rates could be improved controlling the given temperature of top of the condensation chamber. In the distillation tests of the salt wastes containing rare earth compounds, the operation time at a given temperature was greatly reduced changing the given temperature of top of the condensation chamber from 780 to 700 deg C. (author)

Application of molten salt oxidation for the minimization and recovery of plutonium-238 contaminated wastes

Energy Technology Data Exchange (ETDEWEB)

Wishau, R.

1998-05-01

Molten salt oxidation (MSO) is proposed as a {sup 238}Pu waste treatment technology that should be developed for volume reduction and recovery of {sup 238}Pu and as an alternative to the transport and permanent disposal of {sup 238}Pu waste to the WIPP repository. In MSO technology, molten sodium carbonate salt at 800--900 C in a reaction vessel acts as a reaction media for wastes. The waste material is destroyed when injected into the molten salt, creating harmless carbon dioxide and steam and a small amount of ash in the spent salt. The spent salt can be treated using aqueous separation methods to reuse the salt and to recover 99.9% of the precious {sup 238}Pu that was in the waste. Tests of MSO technology have shown that the volume of combustible TRU waste can be reduced by a factor of at least twenty. Using this factor the present inventory of 574 TRU drums of {sup 238}Pu contaminated wastes is reduced to 30 drums. Further {sup 238}Pu waste costs of $22 million are avoided from not having to repackage 312 of the 574 drums to a drum total of more than 4,600 drums. MSO combined with aqueous processing of salts will recover approximately 1.7 kilograms of precious {sup 238}Pu valued at 4 million dollars (at $2,500/gram). Thus, installation and use of MSO technology at LANL will result in significant cost savings compared to present plans to transport and dispose {sup 238}Pu TRU waste to the WIPP site. Using a total net present value cost for the MSO project as $4.09 million over a five-year lifetime, the project can pay for itself after either recovery of 1.6 kg of Pu or through volume reduction of 818 drums or a combination of the two. These savings show a positive return on investment.

Rock salt as a medium for long-term isolation of radioactive wastes - a reassessment

International Nuclear Information System (INIS)

Chaturvedi, L.

1985-01-01

Rock salt has been regarded as a suitable medium for the permanent disposal of high and medium level radioactive wastes since the National Academy of Sciences recommended it in 1957. As a result of detained site-specific studies conducted for the Waste Isolation Pilot Plant (WIPP) project in New Mexico, however, several potential problems which are unique to bedded salt deposits have emerged. These include 1) the need to delineate the extent and rate of past dissolution and projections for the future, 2) the origin and significance of brines often found underlying the salt beds, 3) the rate and volume of migration of brine from the salt crystals towards the heat producing waste canisters, 4) the creep rates and implications for retrievability, and 5) the existence of potash and oil and gas resources with implications of human intrusion in the future. These questions will also be faced for sites in salt domes with added complications due to more complex structure and hydrology. The experience at WIPP shows that the site characterization process for high level waste repositories in bedded or dome salt should aim at identifying the important issues of site suitability early in the process and a clear program should be established to address these issues

Salt Repository Project waste emplacement mode decision paper: Revison 1

International Nuclear Information System (INIS)

1987-08-01

This paper provides a recommendation as to the mode of waste emplacement to be used as the current basis for site characterization activity for the Deaf Smith County, Texas, high level nuclear waste repository site. It also presents a plan for implementing the recommendation so as to provide a high level of confidence in the project's success. Since evaluations of high-level waste disposal in geologic repositories began in the 1950s, most studies emplacement in salt formations employed the vertical orientation for emplacing waste packages in boreholes in the floor of the underground facility. This orientation was used in trials at Project Salt Vault in the 1960s. The Waste Isolation Pilot Plant (WIPP) has recently settled on a combination of vertical and horizontal modes for various waste types. This paper analyzes the information available and develops a project position upon which to base current site characterization activities. The position recommended is that the SRP should continue to use the vertical waste emplacement mode as the reference design and to carry the horizontal mode as a ''passive'' alternative. This position was developed based upon the conclusions of a decision analysis, risk assessment, and cost/schedule impact assessment. 52 refs., 6 figs., 1 tab

Waste form dissolution in bedded salt

International Nuclear Information System (INIS)

Kaufman, A.M.

1980-01-01

A model was devised for waste dissolution in bedded salt, a hydrologically tight medium. For a typical Spent UnReprocessed Fuel (SURF) emplacement, the dissolution rate wll be diffusion limited and will rise to a steady state value after t/sub eq/ approx. = 250 (1+(1-epsilon 0 ) K/sub D//epsilon 0 ) (years) epsilon 0 is the overpack porosity and K/sub d/ is the overpack sorption coefficient. The steady state dissolution rate itself is dominated by the solubility of UO 2 . Steady state rates between 5 x 10 -5 and .5 (g/year) are achievable by SURF emplacements in bedded salt without overpack, and rates between 5 x 10 -7 and 5 x 10 -3 (g/year) with an overpack having porosity of 10 -2

Performance analysis of conceptual waste package designs in salt repositories

International Nuclear Information System (INIS)

Jansen, G. Jr.; Raines, G.E.; Kircher, J.F.

1984-01-01

A performance analysis of commercial high-level waste and spent fuel conceptual package designs in reference repositories in three salt formations was conducted with the WAPPA waste package code. Expected conditions for temperature, stress, brine composition, radiation level, and brine flow rate were used as boundary conditions to compute expected corrosion of a thick-walled overpack of 1025 wrought steel. In all salt formations corrosion by low Mg salt-dissolution brines typical of intrusion scenarios was too slow to cause the package to fail for thousands of years after burial. In high Mg brines judged typical of thermally migrating brines in bedded salt formations, corrosion rates which would otherwise have caused the packages to fail within a few hundred years were limited by brine availability. All of the brine reaching the package was consumed by reaction with the iron in the overpack, thus preventing further corrosion. Uniform brine distribution over the package surface was an important factor in predicting long package lifetimes for the high Mg brines. 14 references, 15 figures

Long-term interactions of full-scale cemented waste simulates with salt brines

Energy Technology Data Exchange (ETDEWEB)

Kienzler, B.; Borkel, C.; Metz, V.; Schlieker, M.

2016-07-01

Since 1967 radioactive wastes have been disposed of in the Asse II salt mine in Northern Germany. A significant part of these wastes originated from the pilot reprocessing plant WAK in Karlsruhe and consisted of cemented NaNO{sub 3} solutions bearing fission products, actinides, as well as process chemicals. With respect to the long-term behavior of these wastes, the licensing authorities requested leaching experiments with full scale samples in relevant salt solutions which were performed since 1979. The experiments aimed at demonstrating the transferability of results obtained with laboratory samples to real waste forms and at the investigation of the effects of the industrial cementation process on the properties of the waste forms. This research program lasted until 2013. The corroding salt solutions were sampled several times and after termination of the experiments, the solid materials were analyzed by various methods. The results presented in this report cover the evolution of the solutions and the chemical and mineralogical characterization of the solids including radionuclides and waste components, and the paragenesis of solid phases (corrosion products). The outcome is compared to the results of model calculations. For safety analysis, conclusions are drawn on radionuclide retention, evolution of the geochemical environment, evolution of the density of solutions, and effects of temperature and porosity of the cement waste simulates on cesium mobilization.

Long-term interactions of full-scale cemented waste simulates with salt brines

International Nuclear Information System (INIS)

Kienzler, B.; Borkel, C.; Metz, V.; Schlieker, M.

2016-01-01

Since 1967 radioactive wastes have been disposed of in the Asse II salt mine in Northern Germany. A significant part of these wastes originated from the pilot reprocessing plant WAK in Karlsruhe and consisted of cemented NaNO 3 solutions bearing fission products, actinides, as well as process chemicals. With respect to the long-term behavior of these wastes, the licensing authorities requested leaching experiments with full scale samples in relevant salt solutions which were performed since 1979. The experiments aimed at demonstrating the transferability of results obtained with laboratory samples to real waste forms and at the investigation of the effects of the industrial cementation process on the properties of the waste forms. This research program lasted until 2013. The corroding salt solutions were sampled several times and after termination of the experiments, the solid materials were analyzed by various methods. The results presented in this report cover the evolution of the solutions and the chemical and mineralogical characterization of the solids including radionuclides and waste components, and the paragenesis of solid phases (corrosion products). The outcome is compared to the results of model calculations. For safety analysis, conclusions are drawn on radionuclide retention, evolution of the geochemical environment, evolution of the density of solutions, and effects of temperature and porosity of the cement waste simulates on cesium mobilization.

Electrodialysis-based separation process for salt recovery and recycling from waste water

Science.gov (United States)

Tsai, Shih-Perng

1997-01-01

A method for recovering salt from a process stream containing organic contaminants is provided, comprising directing the waste stream to a desalting electrodialysis unit so as to create a concentrated and purified salt permeate and an organic contaminants containing stream, and contacting said concentrated salt permeate to a water-splitting electrodialysis unit so as to convert the salt to its corresponding base and acid.

Immobilization of LiCl-Li 2 O pyroprocessing salt wastes in chlorosodalite using glass-bonded hydrothermal and salt-occlusion methods

Energy Technology Data Exchange (ETDEWEB)

Riley, Brian J.; Peterson, Jacob A.; Kroll, Jared O.; Frank, Steven M.

2018-04-01

In this study, salt occlusion and hydrothermal processes were used to make chlorosodalite through reaction with a high-LiCl salt simulating a waste stream following pyrochemical treatment of oxide-based used nuclear fuel. Some products were reacted with glass binders to increase chlorosodalite yield through alkali ion exchange and aide in densification. Hydrothermal processes included reaction of the salt simulant in an acid digestion vessel with either zeolite 4A or sodium aluminate and colloidal silica. Chlorosodalite yields in the crystalline products were nearly complete in the glass-bonded materials at values of 100 mass% for the salt-occlusion method, up to 99.0 mass% for the hydrothermal synthesis with zeolite 4A, and up to 96 mass% for the hydrothermal synthesis with sodium aluminate and colloidal silica. These results show promise for using chemically stable chlorosodalite to immobilize oxide reduction salt wastes.

Immobilization of LiCl-Li2O pyroprocessing salt wastes in chlorosodalite using glass-bonded hydrothermal and salt-occlusion methods

Science.gov (United States)

Riley, Brian J.; Peterson, Jacob A.; Kroll, Jared O.; Frank, Steven M.

2018-04-01

In this study, hydrothermal and salt-occlusion processes were used to make chlorosodalite through reactions with a high-LiCl salt simulating a waste stream generated from pyrochemical treatment of oxide-based used nuclear fuel. Some products were reacted with glass binders to increase chlorosodalite yield through alkali ion exchange and to aid in densification. Hydrothermal processes included reaction of the salt simulant in an autoclave with either zeolite 4A or sodium aluminate and colloidal silica. Chlorosodalite yields in the crystalline products were nearly complete in the glass-bonded materials at values of 100 mass% for the salt-occlusion method, up to 99.0 mass% for the hydrothermal synthesis with zeolite 4A, and up to 96 mass% for the hydrothermal synthesis with sodium aluminate and colloidal silica. These results show promise for using chemically stable chlorosodalite to immobilize oxide reduction salt wastes.

Trails on 18F-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography Leading to Diagnosis of Testicular Adrenal Rest Tumor.

Science.gov (United States)

Kashyap, Raghava

2018-01-01

Testicular adrenal rest tumors (TARTs) are secondary to hypertrophy of adrenal rest cells in the rete testis in settings of hypersecretion of androgens. We present a case of congenital adrenal hyperplasia with TART with clues to the diagnosis on 18 F-fluorodeoxyglucose positron emission tomography/computed tomography ( 18 F-FDG PET/CT). To the best of our knowledge, this is the first reported case on the role of 18 F-FDG PET/CT in TART.

Waste package materials testing for a salt repository: 1983 status summary report

International Nuclear Information System (INIS)

Moak, D.P.

1986-09-01

The United States plans to safely dispose of nuclear waste in deep, stable geologic formations. As part of these plans, the US Department of Energy is sponsoring research on the designing and testing of waste packages and waste package materials. This fiscal year 1983 status report summarizes recent results of waste package materials testing in a salt environment. The results from these tests will be used by waste package designers and performance assessment experts. Release characteristics data are available on two waste forms (spent fuel and waste-containing glass) that were exposed to leaching tests at various radiation levels, temperatures, pH, glass surface area to solution volume ratios, and brine solutions simulating expected salt repository conditions. Candidate materials tested for corrosion resistance and other properties include iron alloys; TI-CODE 12, the most promising titanium alloy for containment; and nickel alloys. In component interaction testing, synergistic effects have not ruled out any candidate material. 21 refs., 37 figs., 15 tabs

Treatment Study Plan for Nitrate Salt Waste Remediation Revision 1.0

Energy Technology Data Exchange (ETDEWEB)

Juarez, Catherine L. [Los Alamos National Lab. (LANL), Los Alamos, NM (United States); Funk, David John [Los Alamos National Lab. (LANL), Los Alamos, NM (United States); Vigil-Holterman, Luciana R. [Los Alamos National Lab. (LANL), Los Alamos, NM (United States); Naranjo, Felicia Danielle [Los Alamos National Lab. (LANL), Los Alamos, NM (United States)

2016-03-07

The two stabilization treatment methods that are to be examined for their effectiveness in the treatment of both the unremediated and remediated nitrate salt wastes include (1) the addition of zeolite and (2) cementation. Zeolite addition is proposed based on the results of several studies and analyses that specifically examined the effectiveness of this process for deactivating nitrate salts. Cementation is also being assessed because of its prevalence as an immobilization method used for similar wastes at numerous facilities around the DOE complex, including at Los Alamos. The results of this Treatment Study Plan will be used to provide the basis for a Resource Conservation and Recovery Act (RCRA) permit modification request of the LANL Hazardous Waste Facility Permit for approval by the New Mexico Environment Department-Hazardous Waste Bureau (NMED-HWB) of the proposed treatment process and the associated facilities.

Influencia de factores perinatales en la pesquisa neonatal de hiperplasia adrenal congénita en Ciudad de La Habana y La Habana Influence of perinatal factors on the neonatal screening of congenital adrenal hyperplasia in Ciudad de La Habana

Directory of Open Access Journals (Sweden)

Tania Mayvel Espinosa Reyes

2012-04-01

congenital adrenal hyperplasia are high. Objectives: to identify the perinatal factors to get involved in the rise and in the normalization time of values of 17 hydroprogesterone (17OHP in patients not involved by a congenital adrenal hyperplasia. Methods: a retrospective, longitudinal and descriptive study was conducted in 1 114 patients from Ciudad de La Habana and La Habana with false-positive results according to screening from January, 2007 to June, 2010. Authors identified the differences in frequency of perinatal factors recognized in this group with a sample of general population, and other including congenital adrenal hyperplasia patients. Results: from the false-positive patients, the 50.7 % was of male sex and the 49.3 % to the female one. The 54.7 % was born by cesarean section and the 82 % has not acute fetal suffering, those small for the gestational age and lower birth weight had mean levels of 17OHP higher. The 68.1 % normalized the OHP at one month of life, independently the type of labor and of the presence of acute fetal suffering but the gestational age and the birth weight had an inverse correlation with the persistence of its rise. There was predominance of eutocia labor in the sick and normal neonates and the cesarean section in the false-positive ones. The mean of gestational age and of the birth weight was significantly minor in the cases of the first group, compared with the remaining groups. Conclusions: the prematurity and the low birth weight had a significant influence on the rise and the persistence of values of 17OHP, but not the type of labor and the acute fetal suffering.

Novel waste printed circuit board recycling process with molten salt

OpenAIRE

Riedewald, Frank; Sousa-Gallagher, Maria

2015-01-01

The objective of the method was to prove the concept of a novel waste PCBs recycling process which uses inert, stable molten salts as the direct heat transfer fluid and, simultaneously, uses this molten salt to separate the metal products in either liquid (solder, zinc, tin, lead, etc.) or solid (copper, gold, steel, palladium, etc.) form at the operating temperatures of 450?470??C. The PCB recovery reactor is essentially a U-shaped reactor with the molten salt providing a continuous fluid, a...

Congenital adrenal hyperplasia: a case report with premature teeth exfoliation and bone resorption.

Science.gov (United States)

Angelopoulou, Matina V; Kontogiorgos, Elias; Emmanouil, Dimitris

2015-06-01

Congenital adrenal hyperplasia (CAH) is an inherited autosomal recessive disorder characterized by insufficient production of cortisol. The aim of this case report was to present a child with CAH, premature exfoliation of primary teeth and accelerated eruption of his permanent teeth related to bone resorption. A 4.5-year-old Caucasian boy with CAH and long-term administration of glucocorticoids was referred for dental restoration. Clinical examination revealed primary molars with worn stainless steel crowns, severe attrition of the upper canines, and absence of the upper incisors. Before the completion of treatment, abnormal mobility of the first upper primary molars and the lower incisors was detected, and a few days later the teeth exfoliated prematurely. Histologic examination revealed normal tooth structure. Alkaline phosphatase and blood cells values were normal. Eruption of the permanent dentition was also accelerated. Tooth mobility was noticed in the permanent teeth as soon as they erupted, along with bone destruction. Examination revealed an elevated level of receptor activator of nuclear factor-κB ligand and lower-than-normal osteoprotegerin and vitamin D levels. The patient was treated with vitamin D supplements, and his teeth have been stable ever since. CAH is a serious chronic disorder appearing in children with accelerated dental development and possibly premature loss of primary teeth. Copyright © 2015 by the American Academy of Pediatrics.

Assessment of tectonic hazards to waste storage in interior-basin salt domes

International Nuclear Information System (INIS)

Kehle, R.

1979-01-01

Salt domes in the northern Gulf of Mexico may make ideal sites for storage of radioactive waste because the area is tectonically quiet. The stability of such salt domes and the tectonic activity are discussed

Clinical, genetic, and structural basis of congenital adrenal hyperplasia due to 11β-hydroxylase deficiency.

Science.gov (United States)

Khattab, Ahmed; Haider, Shozeb; Kumar, Ameet; Dhawan, Samarth; Alam, Dauood; Romero, Raquel; Burns, James; Li, Di; Estatico, Jessica; Rahi, Simran; Fatima, Saleel; Alzahrani, Ali; Hafez, Mona; Musa, Noha; Razzghy Azar, Maryam; Khaloul, Najoua; Gribaa, Moez; Saad, Ali; Charfeddine, Ilhem Ben; Bilharinho de Mendonça, Berenice; Belgorosky, Alicia; Dumic, Katja; Dumic, Miroslav; Aisenberg, Javier; Kandemir, Nurgun; Alikasifoglu, Ayfer; Ozon, Alev; Gonc, Nazli; Cheng, Tina; Kuhnle-Krahl, Ursula; Cappa, Marco; Holterhus, Paul-Martin; Nour, Munier A; Pacaud, Daniele; Holtzman, Assaf; Li, Sun; Zaidi, Mone; Yuen, Tony; New, Maria I

2017-03-07

Congenital adrenal hyperplasia (CAH), resulting from mutations in CYP11B1 , a gene encoding 11β-hydroxylase, represents a rare autosomal recessive Mendelian disorder of aberrant sex steroid production. Unlike CAH caused by 21-hydroxylase deficiency, the disease is far more common in the Middle East and North Africa, where consanguinity is common often resulting in identical mutations. Clinically, affected female newborns are profoundly virilized (Prader score of 4/5), and both genders display significantly advanced bone ages and are oftentimes hypertensive. We find that 11-deoxycortisol, not frequently measured, is the most robust biochemical marker for diagnosing 11β-hydroxylase deficiency. Finally, computational modeling of 25 missense mutations of CYP11B1 revealed that specific modifications in the heme-binding (R374W and R448C) or substrate-binding (W116C) site of 11β-hydroxylase, or alterations in its stability (L299P and G267S), may predict severe disease. Thus, we report clinical, genetic, hormonal, and structural effects of CYP11B1 gene mutations in the largest international cohort of 108 patients with steroid 11β-hydroxylase deficiency CAH.

Low disposal of radioactive wastes in salt formations of the Federal Republic of Germaany

International Nuclear Information System (INIS)

Albrecht, E.

1980-01-01

The salt formations of northern Europe are generally suitable for the storage of radioactive wastes because the region is largely free from earthquakes and the salt formations known as diapires provide effective hydrological sealing. The Federal Republic of Germany employed the Asse Salt Mine of Lower Saxony for research in waste storage. More recently, exploratory work has begun on the construction of a large recycling and disposal plant at the Gorleben salt dome. The geology, hydrology, rock mechanics, and seismicity of the two sites are briefly discussed, including a discussion of experiences gained so far from the Asse site. 11 refs

Removal of salt from high-level waste tanks by density-driven circulation or mechanical agitation

International Nuclear Information System (INIS)

Kiser, D.L.

1981-01-01

Twenty-two high-level waste storage tanks at the Savannah River Plant are to be retired in the tank replacement/waste transfer program. The salt-removal portion of this program requires dissolution of about 19 million liters of salt cake. Steam circulation jets were originally proposed to dissolve the salt cake. However, the jets heated the waste tank to 80 to 90 0 C. This high temperature required a long cooldown period before transfer of the supernate by jet, and increased the risk of stress-corrosion cracking in these older tanks. A bench-scale investigation at the Savannah River Laboratory developed two alternatives to steam-jet circulation. One technique was density-driven circulation, which in bench tests dissolved salt at the same rate as a simulated steam circulation jet but at a lower temperature. The other technique was mechanical agitation, which dissolved the salt cake faster and required less fresh water than either density-driven circulation or the simulated steam circulation jet. Tests in an actual waste tank verified bench-scale results and demonstrated the superiority of mechanical agitation

Molt salts reactors capacity for wastes incineration and energy production

International Nuclear Information System (INIS)

David, S.; Nuttin, A.

2005-01-01

The molten salt reactors present many advantages in the framework of the IV generation systems development for the energy production and/or the wastes incineration. After a recall of the main studies realized on the molten salt reactors, this document presents the new concepts and the identified research axis: the MSRE project and experience, the incinerators concepts, the thorium cycle. (A.L.B.)

Considerations of the Differences between Bedded and Domal Salt Pertaining to Disposal of Heat-Generating Nuclear Waste

Energy Technology Data Exchange (ETDEWEB)

Hansen, Francis D. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Kuhlman, Kristopher L. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Sobolik, Steven R. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States)

2016-07-07

Salt formations hold promise for eternal removal of nuclear waste from our biosphere. Germany and the United States have ample salt formations for this purpose, ranging from flat-bedded formations to geologically mature dome structures. As both nations revisit nuclear waste disposal options, the choice between bedded, domal, or intermediate pillow formations is once again a contemporary issue. For decades, favorable attributes of salt as a disposal medium have been extoled and evaluated, carefully and thoroughly. Yet, a sense of discovery continues as science and engineering interrogate naturally heterogeneous systems. Salt formations are impermeable to fluids. Excavation-induced fractures heal as seal systems are placed or natural closure progresses toward equilibrium. Engineering required for nuclear waste disposal gains from mining and storage industries, as humans have been mining salt for millennia. This great intellectual warehouse has been honed and distilled, but not perfected, for all nuances of nuclear waste disposal. Nonetheless, nations are able and have already produced suitable license applications for radioactive waste disposal in salt. A remaining conundrum is site location. Salt formations provide isolation and geotechnical barriers reestablish impermeability after waste is placed in the geology. Between excavation and closure, physical, mechanical, thermal, chemical, and hydrological processes ensue. Positive attributes for isolation in salt have many commonalities independent of the geologic setting. In some cases, specific details of the environment will affect the disposal concept and thereby define interaction of features, events and processes, while simultaneously influencing scenario development. Here we identify and discuss high-level differences and similarities of bedded and domal salt formations. Positive geologic and engineering attributes for disposal purposes are more common among salt formations than are significant differences

Considerations of the Differences between Bedded and Domal Salt Pertaining to Disposal of Heat-Generating Nuclear Waste

Energy Technology Data Exchange (ETDEWEB)

Hansen, Francis D. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Kuhlman, Kristopher L. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Sobolik, Steven R. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States)

2016-07-07

Salt formations hold promise for eternal removal of nuclear waste from our biosphere. Germany and the United States have ample salt formations for this purpose, ranging from flat-bedded formations to geologically mature dome structures. As both nations revisit nuclear waste disposal options, the choice between bedded, domal, or intermediate pillow formations is once again a contemporary issue. For decades, favorable attributes of salt as a disposal medium have been extoled and evaluated, carefully and thoroughly. Yet, a sense of discovery continues as science and engineering interrogate naturally heterogeneous systems. Salt formations are impermeable to fluids. Excavation-induced fractures heal as seal systems are placed or natural closure progresses toward equilibrium. Engineering required for nuclear waste disposal gains from mining and storage industries, as humans have been mining salt for millennia. This great intellectual warehouse has been honed and distilled, but not perfected, for all nuances of nuclear waste disposal. Nonetheless, nations are able and have already produced suitable license applications for radioactive waste disposal in salt. A remaining conundrum is site location. Salt formations provide isolation, and geotechnical barriers reestablish impermeability after waste is placed in the geology. Between excavation and closure, physical, mechanical, thermal, chemical, and hydrological processes ensue. Positive attributes for isolation in salt have many commonalities independent of the geologic setting. In some cases, specific details of the environment will affect the disposal concept and thereby define interaction of features, events and processes, while simultaneously influencing scenario development. Here we identify and discuss high-level differences and similarities of bedded and domal salt formations. Positive geologic and engineering attributes for disposal purposes are more common among salt formations than are significant differences

Considerations of the Differences between Bedded and Domal Salt Pertaining to Disposal of Heat-Generating Nuclear Waste

International Nuclear Information System (INIS)

Hansen, Francis D.; Kuhlman, Kristopher L.; Sobolik, Steven R.

2016-01-01

Salt formations hold promise for eternal removal of nuclear waste from our biosphere. Germany and the United States have ample salt formations for this purpose, ranging from flat-bedded formations to geologically mature dome structures. As both nations revisit nuclear waste disposal options, the choice between bedded, domal, or intermediate pillow formations is once again a contemporary issue. For decades, favorable attributes of salt as a disposal medium have been extoled and evaluated, carefully and thoroughly. Yet, a sense of discovery continues as science and engineering interrogate naturally heterogeneous systems. Salt formations are impermeable to fluids. Excavation-induced fractures heal as seal systems are placed or natural closure progresses toward equilibrium. Engineering required for nuclear waste disposal gains from mining and storage industries, as humans have been mining salt for millennia. This great intellectual warehouse has been honed and distilled, but not perfected, for all nuances of nuclear waste disposal. Nonetheless, nations are able and have already produced suitable license applications for radioactive waste disposal in salt. A remaining conundrum is site location. Salt formations provide isolation, and geotechnical barriers reestablish impermeability after waste is placed in the geology. Between excavation and closure, physical, mechanical, thermal, chemical, and hydrological processes ensue. Positive attributes for isolation in salt have many commonalities independent of the geologic setting. In some cases, specific details of the environment will affect the disposal concept and thereby define interaction of features, events and processes, while simultaneously influencing scenario development. Here we identify and discuss high-level differences and similarities of bedded and domal salt formations. Positive geologic and engineering attributes for disposal purposes are more common among salt formations than are significant differences

Using Aspen simulation package to determine solubility of mixed salts in TRU waste evaporator bottoms

Energy Technology Data Exchange (ETDEWEB)

Hatchell, J.L.

1998-03-01

Nitric acid from plutonium process waste is a candidate for waste minimization by recycling. Process simulation software packages, such as Aspen, are valuable tools to estimate how effective recovery processes can be, however, constants in equations of state for many ionic components are not in their data libraries. One option is to combine single salt solubility`s in the Aspen model for mixed salt system. Single salt solubilities were regressed in Aspen within 0.82 weight percent of literature values. These were combined into a single Aspen model and used in the mixed salt studies. A simulated nitric acid waste containing mixed aluminum, calcium, iron, magnesium and sodium nitrate was tested to determine points of solubility between 25 and 100 C. Only four of the modeled experimental conditions, at 50 C and 75 C, produced a saturated solution. While experimental results indicate that sodium nitrate is the first salt to crystallize out, the Aspen computer model shows that the most insoluble salt, magnesium nitrate, the first salt to crystallize. Possible double salt formation is actually taking place under experimental conditions, which is not captured by the Aspen model.

Radioactive waste isolation in salt: geochemistry of brine in rock salt in temperature gradients and gamma-radiation fields - a selective annotated bibliography

International Nuclear Information System (INIS)

Hull, A.B.; Williams, L.B.

1985-07-01

Evaluation of the extensive research concerning brine geochemistry and transport is critically important to successful exploitation of a salt formation for isolating high-level radioactive waste. This annotated bibliography has been compiled from documents considered to provide classic background material on the interactions between brine and rock salt, as well as the most important results from more recent research. Each summary elucidates the information or data most pertinent to situations encountered in siting, constructing, and operating a mined repository in salt for high-level radioactive waste. The research topics covered include the basic geology, depositional environment, mineralogy, and structure of evaporite and domal salts, as well as fluid inclusions, brine chemistry, thermal and gamma-radiation effects, radionuclide migration, and thermodynamic properties of salts and brines. 4 figs., 6 tabs

Radioactive waste isolation in salt: geochemistry of brine in rock salt in temperature gradients and gamma-radiation fields - a selective annotated bibliography

Energy Technology Data Exchange (ETDEWEB)

Hull, A.B.; Williams, L.B.

1985-07-01

Evaluation of the extensive research concerning brine geochemistry and transport is critically important to successful exploitation of a salt formation for isolating high-level radioactive waste. This annotated bibliography has been compiled from documents considered to provide classic background material on the interactions between brine and rock salt, as well as the most important results from more recent research. Each summary elucidates the information or data most pertinent to situations encountered in siting, constructing, and operating a mined repository in salt for high-level radioactive waste. The research topics covered include the basic geology, depositional environment, mineralogy, and structure of evaporite and domal salts, as well as fluid inclusions, brine chemistry, thermal and gamma-radiation effects, radionuclide migration, and thermodynamic properties of salts and brines. 4 figs., 6 tabs.

Adrenal Incidentalomas with Supraphysiologic Response to ACTH Stimulus: A Case Report

Directory of Open Access Journals (Sweden)

Marianna Antonopoulou

2012-01-01

Full Text Available We present the diagnostic approach of a patient with adrenal incidentalomas. A 72-year-old African American male had a CT scan of the abdomen showing right and left adrenal masses measuring and , respectively. The patient had negative hormonal workup. The radiologist insisted that the CT findings are consistent with adrenal hyperplasia, and therefore he underwent ACTH stimulation to rule out late-onset congenital adrenal hyperplasia (CAH. The stimulation test revealed that 17-hydroxyprogesterone and 11-deoxycortisol increased to levels high enough to confirm CAH, but cortisol had exaggerated response as well, thus making the diagnosis of CAH unlikely where metabolism is shifted to precursors. Subsequently, the patient underwent screening for Cushing's syndrome (CS with a dexamethasone suppression test. Patient failed the suppresion test, raising the issue for subclinical CS (SCS, likely due to ACTH-independent macronodular adrenal hyperplasia. Our patient had been diagnosed with MGUS and so far there are only 3 case reports of extramedullary plasmacytoma arising from the adrenals. One was bilateral and one had functional abnormalities. Our differential diagnosis includes subclinical CS with aberrant receptors versus a functioning extramedullary plasmacytoma.

Test procedures for polyester immobilized salt-containing surrogate mixed wastes

International Nuclear Information System (INIS)

Biyani, R.K.; Hendrickson, D.W.

1997-01-01

These test procedures are written to meet the procedural needs of the Test Plan for immobilization of salt containing surrogate mixed waste using polymer resins, HNF-SD-RE-TP-026 and to ensure adequacy of conduct and collection of samples and data. This testing will demonstrate the use of four different polyester vinyl ester resins in the solidification of surrogate liquid and dry wastes, similar to some mixed wastes generated by DOE operations

User's manual and guide to SALT3 and SALT4: two-dimensional computer codes for analysis of test-scale underground excavations for the disposal of radioactive waste in bedded salt deposits

International Nuclear Information System (INIS)

Lindner, E.N.; St John, C.M.; Hart, R.D.

1984-02-01

SALT3 and SALT4 are two-dimensional analytical/displacement-discontinuity codes designed to evaluate temperatures, deformation, and stresses associated with underground disposal of radioactive waste in bedded salt. These codes were developed by the University of Minnesota for the Office of Nuclear Waste Isolation in 1979. The present documentation describes the mathematical equations of the physical system being modeled, the numerical techniques utilized, and the organization of these computer codes. The SALT3 and SALT4 codes can simulate: (a) viscoelastic behavior in pillars adjacent to excavations; (b) transversely isotropic elastic moduli such as those exhibited by bedded or stratified rock; and (c) excavation sequence. Major advantages of these codes are: (a) computational efficiency; (b) the small amount of input data required; and (c) a creep law based on laboratory experimental data for salt. The main disadvantage is that some of the assumptions in the formulation of the codes, i.e., the homogeneous elastic half-space and temperature-independent material properties, render it unsuitable for canister-scale analysis or analysis of lateral deformation of the pillars. The SALT3 and SALT4 codes can be used for parameter sensitivity analyses of two-dimensional, repository-scale, thermomechanical response in bedded salt during the excavation, operational, and post-closure phases. It is especially useful in evaluating alternative patterns and sequences of excavation or waste canister placement. SALT3 is a refinement of an earlier code, SALT, and includes a fully anelastic creep model and thermal stress routine. SALT4 is a later version, and incorporates a revised creep model which is strain-hardening

Hydrological methods preferentially recover cesium from nuclear waste salt cake

International Nuclear Information System (INIS)

Brooke, J.N.; Hamm, L.L.

1997-01-01

The Savannah River Site is treating high level radioactive waste in the form of insoluble solids (sludge), crystallized salt (salt cake), and salt solutions. High costs and operational concerns have prompted DOE to look for ways to improve the salt cake treatment process. A numerical model was developed to evaluate the feasibility of pump and treat technology for extracting cesium from salt cake. A modified version of the VAM3DCG code was used to first establish a steady-state flow field, then to simulate 30 days of operation. Simulation results suggest that efficient cesium extraction can be obtained with low displacement volumes. The actual extraction process will probably be less impressive because of nonuniform properties. 2 refs., 2 figs

Risk assessment of nonhazardous oil-field waste disposal in salt caverns.

Energy Technology Data Exchange (ETDEWEB)

Elcock, D.

1998-03-05

In 1996, Argonne National Laboratory (ANL) conducted a preliminary technical and legal evaluation of disposing of nonhazardous oil-field wastes (NOW) into salt caverns. Argonne determined that if caverns are sited and designed well, operated carefully, closed properly, and monitored routinely, they could be suitable for disposing of oil-field wastes. On the basis of these findings, Argonne subsequently conducted a preliminary evaluation of the possibility that adverse human health effects (carcinogenic and noncarcinogenic) could result from exposure to contaminants released from the NOW disposed of in domal salt caverns. Steps used in this evaluation included the following: identifying potential contaminants of concern, determining how humans could be exposed to these contaminants, assessing contaminant toxicities, estimating contaminant intakes, and calculating human cancer and noncancer risk estimates. Five postclosure cavern release scenarios were assessed. These were inadvertent cavern intrusion, failure of the cavern seal, failure of the cavern through cracks, failure of the cavern through leaky interbeds, and a partial collapse of the cavern roof. Assuming a single, generic, salt cavern and generic oil-field wastes, potential human health effects associated with constituent hazardous substances (arsenic, benzene, cadmium, and chromium) were assessed under each of these scenarios. Preliminary results provided excess cancer risk and hazard index (referring to noncancer health effects) estimates that were well within the US Environmental Protection Agency (EPA) target range for acceptable exposure risk levels. These results led to the preliminary conclusion that from a human health perspective, salt caverns can provide an acceptable disposal method for nonhazardous oil-field wastes.

Risk assessment of nonhazardous oil-field waste disposal in salt caverns

International Nuclear Information System (INIS)

Elcock, D.

1998-01-01

In 1996, Argonne National Laboratory (ANL) conducted a preliminary technical and legal evaluation of disposing of nonhazardous oil-field wastes (NOW) into salt caverns. Argonne determined that if caverns are sited and designed well, operated carefully, closed properly, and monitored routinely, they could be suitable for disposing of oil-field wastes. On the basis of these findings, Argonne subsequently conducted a preliminary evaluation of the possibility that adverse human health effects (carcinogenic and noncarcinogenic) could result from exposure to contaminants released from the NOW disposed of in domal salt caverns. Steps used in this evaluation included the following: identifying potential contaminants of concern, determining how humans could be exposed to these contaminants, assessing contaminant toxicities, estimating contaminant intakes, and calculating human cancer and noncancer risk estimates. Five postclosure cavern release scenarios were assessed. These were inadvertent cavern intrusion, failure of the cavern seal, failure of the cavern through cracks, failure of the cavern through leaky interbeds, and a partial collapse of the cavern roof. Assuming a single, generic, salt cavern and generic oil-field wastes, potential human health effects associated with constituent hazardous substances (arsenic, benzene, cadmium, and chromium) were assessed under each of these scenarios. Preliminary results provided excess cancer risk and hazard index (referring to noncancer health effects) estimates that were well within the US Environmental Protection Agency (EPA) target range for acceptable exposure risk levels. These results led to the preliminary conclusion that from a human health perspective, salt caverns can provide an acceptable disposal method for nonhazardous oil-field wastes

Protein removal from waste brines generated during ham salting through acidification and centrifugation.

Science.gov (United States)

Gutiérrez-Martínez, Maria del Rosario; Muñoz-Guerrero, Hernán; Alcaína-Miranda, Maria Isabel; Barat, José Manuel

2014-03-01

The salting step in food processes implies the production of large quantities of waste brines, having high organic load, high conductivity, and other pollutants with high oxygen demand. Direct disposal of the residual brine implies salinization of soil and eutrophication of water. Since most of the organic load of the waste brines comes from proteins leaked from the salted product, precipitation of dissolved proteins by acidification and removal by centrifugation is an operation to be used in waste brine cleaning. The aim of this study is optimizing the conditions for carrying out the separation of proteins from waste brines generated in the pork ham salting operation, by studying the influence of pH, centrifugal force, and centrifugation time. Models for determining the removal of proteins depending on the pH, centrifugal force, and time were obtained. The results showed a high efficacy of the proposed treatment for removing proteins, suggesting that this method could be used for waste brine protein removal. The best pH value to be used in an industrial process seems to be 3, while the obtained results indicate that almost 90% of the proteins from the brine can be removed by acidification followed by centrifugation. A further protein removal from the brine should have to be achieved using filtrating techniques, which efficiency could be highly improved as a consequence of the previous treatment through acidification and centrifugation. Waste brines from meat salting have high organic load and electrical conductivity. Proteins can be removed from the waste brine by acidification and centrifugation. The total protein removal can be up to 90% of the initial content of the waste brine. Protein removal is highly dependent on pH, centrifugation rate, and time. © 2014 Institute of Food Technologists®

Thermal decomposition of nitrate salts liquid waste for the lagoon sludge treatment

International Nuclear Information System (INIS)

Hwang, D. S.; Oh, J. H.; Kim, Y. K.; Lee, K. Y.; Choi, Y. D.; Hwang, S. T.; Park, J. H.

2004-01-01

This study investigated the thermal decomposition property of nitrate salts liquid waste which is produced in a series of the processes for the sludge treatment. Thermal decomposition property was analyzed by TG/DTA and XRD. Most ammonium nitrate in the nitrate salts liquid waste was decomposed at 250 .deg. C and calcium nitrate was decomposed and converted into calcium oxide at 550 .deg. C. Sodium nitrate was decomposed at 700 .deg. C and converted into sodium oxide which reacts with water easily. But sodium oxide was able to convert into a stable compound by adding alumina. Therefore, nitrate salts liquid waste can be treated by two steps as follows. First, ammonium nitrate is decomposed at 250 .deg. C. Second, alumina is added in residual solid sodium nitrate and calcium nitrate and these are decomposed at 900 .deg. C. Final residue consists of calcium oxide and Na 2 O.Al 2 O 3 and can be stored stably

Modeling of Sulfate Double-Salt in Nuclear Wastes

International Nuclear Information System (INIS)

Toghiani, B.; Lindner, J.S.; Weber, C.F.; Hunt, R.D.

2000-01-01

The Environmental Simulation Program (ESP) continues to adequately predict the solubility of most key chemical systems in the Hanford tank waste. For example, the ESP predictions were in fair agreement with the solubility experiments for the fluoride-phosphate system, although ESP probably underestimates the aqueous amounts. Due to the importance of this system in the formation of pipeline plugs, additional experiments have been made at elevated temperatures, and improvements to the ESP database will be made. ESP encountered problems with sulfate systems because the Public database for ESP does not include anhydrous sodium sulfate in mixed solutions below 32.4 C. This limitation leads to convergence problems and to spurious predictions of solubility near the transition point with sodium sulfate decahydrate when other salts such as sodium nitrate are present. However, ESP was able to make reasonable solubility predictions with a corrected database, demonstrating the need to validate and document the various databases that can be used by ESP. Even though ESP does not include the sulfate-nitrate double salt, this omission does not appear to be a major problem. The solubility predictions with and without the sulfate-nitrate double salt are comparable. In sharp contrast, the sulfate-fluoride double salt is included, but ESP still underestimates solubility in some cases. This problem can misrepresent the ionic strength of the solution, which is an important factor in the formation of pipeline plugs. Solubility tests on the sulfate-fluoride system are planned to provide additional data at higher temperatures and in caustic solutions. These results will be used to improve the range and accuracy of ESP predictions. ESP will continue to provide important predictions for waste processing operations while being evaluated and improved. For example, ESP will be used to determine the amount of water for the saltcake dissolution efforts at Hanford. When ESP underestimates the

Pure Androgen-Secreting Adrenal Adenoma Associated with Resistant Hypertension

Directory of Open Access Journals (Sweden)

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.

Transient Pseudohypoaldosteronism due to Urinary Tract Infection in Infancy: A Report of 4 Cases

Directory of Open Access Journals (Sweden)

Kaplowitz Paul

2009-05-01

Full Text Available Hyponatremia with hyperkalemia in infancy is an uncommon but life-threatening occurrence. In the first weeks of life, this scenario is often associated with aldosterone deficiency due to salt-wasting congenital adrenal hyperplasia. However, alternative diagnoses involving inadequate mineralocorticoid secretion or action must be considered, particularly for infants one month of age or older. We report four infants who presented with profound hyponatremia accompanied by urinary tract infection, ultimately leading to the diagnosis of transient pseudohypoaldosteronism. Our cases provide support for the idea that the renal tubular resistance to aldosterone is due to urinary tract infection itself rather than to underlying urinary tract anomalies typically found in these infants. Awareness of this condition is important so that serum aldosterone, urine sodium, and urine cultures may be obtained immediately in any infant presenting with hyponatremia and hyperkalemia in whom a diagnosis of congenital adrenal hyperplasia was not found. Adequate replacement with intravenous saline and antibiotic therapy is sufficient to correct sodium levels over 24–48 hours.

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...... 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....

Conceptual waste package interim product specifications and data requirements for disposal of borosilicate glass defense high-level waste forms in salt geologic repositories

International Nuclear Information System (INIS)

1983-06-01

The conceptual waste package interim product specifications and data requirements presented are applicable specifically to the normal borosilicate glass product of the Defense Waste Processing Facility (DWPF). They provide preliminary numerical values for the defense high-level waste form parameters and properties identified in the waste form performance specification for geologic isolation in salt repositories. Subject areas treated include containment and isolation, operational period safety, criticality control, waste form/production canister identification, and waste package performance testing requirements. This document was generated for use in the development of conceptual waste package designs in salt. It will be revised as additional data, analyses, and regulatory requirements become available

Brine migration in salt and its implications in the geologic disposal of nuclear waste

International Nuclear Information System (INIS)

Jenks, G.H.; Claiborne, H.C.

1981-12-01

This report respresents a comprehensive review and analysis of available information relating to brine migration in salt surrounding radioactive waste in a salt repository. The topics covered relate to (1) the characteristics of salt formations and waste packages pertinent to considerations of rates, amounts, and effects of brine migration, (2) experimental and theoretical information on brine migration, and (3) means of designing to minimize any adverse effects of brine migration. Flooding, brine pockets, and other topics were not considered, since these features will presumably be eliminated by appropriate site selection and repository design. 115 references

Options Assessment Report: Treatment of Nitrate Salt Waste at Los Alamos National Laboratory

Energy Technology Data Exchange (ETDEWEB)

Robinson, Bruce Alan [Los Alamos National Lab. (LANL), Los Alamos, NM (United States); Stevens, Patrice Ann [Los Alamos National Lab. (LANL), Los Alamos, NM (United States)

2015-12-17

This report documents the methodology used to select a method of treatment for the remediated nitrate salt (RNS) and unremediated nitrate salt (UNS) waste containers at Los Alamos National Laboratory (LANL). The method selected should treat the containerized waste in a manner that renders the waste safe and suitable for transport and final disposal in the Waste Isolation Pilot Plant (WIPP) repository, under specifications listed in the WIPP Waste Acceptance Criteria (DOE/CBFO, 2013). LANL recognizes that the results must be thoroughly vetted with the New Mexico Environment Department (NMED) and that a modification to the LANL Hazardous Waste Facility Permit is a necessary step before implementation of this or any treatment option. Likewise, facility readiness and safety basis approvals must be received from the Department of Energy (DOE). This report presents LANL’s preferred option, and the documentation of the process for reaching the recommended treatment option for RNS and UNS waste, and is presented for consideration by NMED and DOE.

Options assessment report: Treatment of nitrate salt waste at Los Alamos National Laboratory

Energy Technology Data Exchange (ETDEWEB)

Robinson, Bruce Alan [Los Alamos National Lab. (LANL), Los Alamos, NM (United States); Stevens, Patrice Ann [Los Alamos National Lab. (LANL), Los Alamos, NM (United States)

2015-09-16

This report documents the methodology used to select a method of treatment for the remediated nitrate salt (RNS) and unremediated nitrate salt (UNS) waste containers at Los Alamos National Laboratory (LANL). The method selected should treat the containerized waste in a manner that renders the waste safe and suitable for transport and final disposal in the Waste Isolation Pilot Plant (WIPP) repository, under specifications listed in the WIPP Waste Acceptance Criteria (DOE/CBFO, 2013). LANL recognized that the results must be thoroughly vetted with the New Mexico Environment Department (NMED) and the a modification to the LANL Hazardous Waste Facility Permit is a necessary step before implementation of this or any treatment option. Likewise, facility readiness and safety basis approvals must be received from the Department of Energy (DOE). This report presents LANL's preferred option, and the documentation of the process for reaching the recommended treatment option for RNS and UNS waste, and is presented for consideration by NMED and DOE.

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.

Relative adrenal insufficiency in post-transplant lymphoproliferative disorder.

Directory of Open Access Journals (Sweden)

Cinclair R

2003-01-01

Full Text Available Post-transplant lymphoproliferative disorder is treated with rapid decrement of immunosuppressive therapy. This cannot be achieved with ease in patients on long-term glucocorticoid therapy, as chronically suppressed adrenal glands may not be capable of mounting adequate response to stress. A 52-year-old Caucasian male presented with fever, orthostatic hypotension, lymphadenopathy and hyponatraemia. Serum cortisol levels were within normal levels with a sub optimal response to stimulation by ACTH. Hyponatraemia and orthostasis responded poorly to fluid restriction, saline and salt repletion but corrected after increasing the steroid dose. The normal baseline cortisol levels represented a stimulated adrenal gland, however, the ACTH stimulation had inadequate response. This sub optimal stimulation and a good response to increased steroids suggest the presence of relative or occult adrenal insufficiency. Relative adrenal insufficiency must be considered in patients who have received prolonged glucocorticoid therapy and have symptoms such as hypotension and/or hyponatraemia.

Assessment of lead tellurite glass for immobilizing electrochemical salt wastes from used nuclear fuel reprocessing

Energy Technology Data Exchange (ETDEWEB)

Riley, Brian J.; Kroll, Jared O.; Peterson, Jacob A.; Pierce, David A.; Ebert, William L.; Williams, Benjamin D.; Snyder, Michelle M. V.; Frank, Steven M.; George, Jaime L.; Kruska, Karen

2017-11-01

This paper provides an overview of research evaluating the use of tellurite glass as a waste form for salt wastes from electrochemical processing. The capacities to immobilize different salts were evaluated including: a LiCl-Li2O oxide reduction salt (for oxide fuel) containing fission products, a LiCl-KCl eutectic salt (for metallic fuel) containing fission products, and SrCl2. Physical and chemical properties of the glasses were characterized by using X-ray diffraction, bulk density measurements, chemical durability tests, scanning electron microscopy, and energy dispersive X-ray emission spectroscopy. These glasses were found to accommodate high concentrations of halide salts and have high densities. However, improvements are needed to meet chemical durability requirements.

Conceptual waste package interim product specifications and data requirements for disposal of glass commercial high-level waste forms in salt geologic repositories

International Nuclear Information System (INIS)

1983-10-01

The conceptual waste package interim product specifications and data requirements presented are applicable to the reference glass composition described in PNL-3838 and carbon steel canister described in ONWI-438. They provide preliminary numerical values for the commercial high-level waste form parameters and properties identified in the waste form performance specification for geologic isolation in salt repositories. Subject areas treated include containment and isolation, operational period safety, criticality control, waste form/production canister identification, and waste package performance testing requirements. This document was generated for use in the development of conceptual waste package designs in salt. It will be revised as additional data, analyses and regulatory requirements become available. 13 references, 1 figure

Risk analyses for disposing nonhazardous oil field wastes in salt caverns

Energy Technology Data Exchange (ETDEWEB)

Tomasko, D.; Elcock, D.; Veil, J.; Caudle, D.

1997-12-01

Salt caverns have been used for several decades to store various hydrocarbon products. In the past few years, four facilities in the US have been permitted to dispose nonhazardous oil field wastes in salt caverns. Several other disposal caverns have been permitted in Canada and Europe. This report evaluates the possibility that adverse human health effects could result from exposure to contaminants released from the caverns in domal salt formations used for nonhazardous oil field waste disposal. The evaluation assumes normal operations but considers the possibility of leaks in cavern seals and cavern walls during the post-closure phase of operation. In this assessment, several steps were followed to identify possible human health risks. At the broadest level, these steps include identifying a reasonable set of contaminants of possible concern, identifying how humans could be exposed to these contaminants, assessing the toxicities of these contaminants, estimating their intakes, and characterizing their associated human health risks. The contaminants of concern for the assessment are benzene, cadmium, arsenic, and chromium. These were selected as being components of oil field waste and having a likelihood to remain in solution for a long enough time to reach a human receptor.

Bone mineral density in young adult women with congenital adrenal hyperplasia

Directory of Open Access Journals (Sweden)

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.

Hydrometallurgical treatment of plutonium. Bearing salt baths waste

International Nuclear Information System (INIS)

Bros, P.; Gozlan, J.P.; Lecomte, M.; Bourges, J.

1993-01-01

The salt flux issuing from the electrorefining of plutonium metal alloy in salt baths (KCI + NaCI) poses a difficult problem of the back-end alpha waste management. An alternative to the salt process promoted by Los Alamos Laboratory is to develop a hydrometallurgical treatment. A new process based on the electrochemistry technique in aqueous solution has been defined and tested successfully in the CEA. The diagram of the process exhibits two principal steps: in the head-end, a dissolution in HNO 3 medium accompanied with an electrolytic dechlorination leading to a quantitative elimination of chloride as CI 2 gas followed by its trapping one soda lime cartridge, a complete oxidative dissolution of the refractory Pu residues by electrogenerated Ag(II), in the back-end: the Pu and Am recoveries by chromatographic extractions. (authors). 10 figs., 9 refs

GATA transcription factors in testicular adrenal rest tumours

Directory of Open Access Journals (Sweden)

Manon Engels

2017-11-01

Full Text Available 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.

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.

Molten salt combustion of radioactive wastes

International Nuclear Information System (INIS)

Grantham, L.F.; McKenzie, D.E.; Richards, W.L.; Oldenkamp, R.D.

1976-01-01

The Atomics International Molten Salt Combustion Process reduces the weight and volume of combustible β-γ contaminated transuranic waste by utilizing air in a molten salt medium to combust organic materials, to trap particulates, and to react chemically with any acidic gases produced during combustion. Typically, incomplete combustion products such as hydrocarbons and carbon monoxide are below detection limits (i.e., 3 ) is directly related to the sodium chloride vapor pressure of the melt; >80% of the particulate is sodium chloride. Essentially all metal oxides (combustion ash) are retained in the melt, e.g., >99.9% of the plutonium, >99.6% of the europium, and >99.9% of the ruthenium are retained in the melt. Both bench-scale radioactive and pilot scale (50 kg/hr) nonradioactive combustion tests have been completed with essentially the same results. Design of three combustors for industrial applications are underway

Alternative Electrochemical Salt Waste Forms, Summary of FY/CY2011 Results

International Nuclear Information System (INIS)

Riley, Brian J.; McCloy, John S.; Crum, Jarrod V.; Rodriguez, Carmen P.; Windisch, Charles F.; Lepry, William C.; Matyas, Josef; Westman, Matthew P.; Rieck, Bennett T.; Lang, Jesse B.; Pierce, David A.

2011-01-01

This report summarizes the 2011 fiscal+calendar year efforts for developing waste forms for a spent salt generated in reprocessing nuclear fuel with an electrochemical separations process. The two waste forms are tellurite (TeO2-based) glasses and sol-gel-derived high-halide mineral analogs to stable minerals found in nature.

Alternative Electrochemical Salt Waste Forms, Summary of FY/CY2011 Results

Energy Technology Data Exchange (ETDEWEB)

Riley, Brian J.; McCloy, John S.; Crum, Jarrod V.; Rodriguez, Carmen P.; Windisch, Charles F.; Lepry, William C.; Matyas, Josef; Westman, Matthew P.; Rieck, Bennett T.; Lang, Jesse B.; Pierce, David A.

2011-12-01

This report summarizes the 2011 fiscal+calendar year efforts for developing waste forms for a spent salt generated in reprocessing nuclear fuel with an electrochemical separations process. The two waste forms are tellurite (TeO2-based) glasses and sol-gel-derived high-halide mineral analogs to stable minerals found in nature.

Possible salt mine sites for radioactive waste disposal in the northeastern states

Energy Technology Data Exchange (ETDEWEB)

Landes, K.K.

1972-06-30

The motivation for this investigation is the necessity for finding the safest possible repository for solid atomic plant wastes. It is believed that rooms mined in thick beds of salt would afford the best sanctuary. This is due especially to the impermeability of massive rock salt. This rock has enough plasticity so that it tends to give rather than fracture when disturbed by movements of the earth's crust. In addition, due to water conditions at the time of deposition, the rocks most commonly associated with salt (anhydrite and shale) are likewise relatively impervious. A number of areas have been selected for detailed discussion because of the excellence of the geological and environmental factors. The optimum requirements for a viable waste disposal prospect are described in detail and nine prospects are considered further.

Possible salt mine sites for radioactive waste disposal in the northeastern states

International Nuclear Information System (INIS)

Landes, K.K.

1972-01-01

The motivation for this investigation is the necessity for finding the safest possible repository for solid atomic plant wastes. It is believed that rooms mined in thick beds of salt would afford the best sanctuary. This is due especially to the impermeability of massive rock salt. This rock has enough plasticity so that it tends to give rather than fracture when disturbed by movements of the earth's crust. In addition, due to water conditions at the time of deposition, the rocks most commonly associated with salt (anhydrite and shale) are likewise relatively impervious. A number of areas have been selected for detailed discussion because of the excellence of the geological and environmental factors. The optimum requirements for a viable waste disposal prospect are described in detail and nine prospects are considered further

Hydrous mineral dehydration around heat-generating nuclear waste in bedded salt formations.

Science.gov (United States)

Jordan, Amy B; Boukhalfa, Hakim; Caporuscio, Florie A; Robinson, Bruce A; Stauffer, Philip H

2015-06-02

Heat-generating nuclear waste disposal in bedded salt during the first two years after waste emplacement is explored using numerical simulations tied to experiments of hydrous mineral dehydration. Heating impure salt samples to temperatures of 265 °C can release over 20% by mass of hydrous minerals as water. Three steps in a series of dehydration reactions are measured (65, 110, and 265 °C), and water loss associated with each step is averaged from experimental data into a water source model. Simulations using this dehydration model are used to predict temperature, moisture, and porosity after heating by 750-W waste canisters, assuming hydrous mineral mass fractions from 0 to 10%. The formation of a three-phase heat pipe (with counter-circulation of vapor and brine) occurs as water vapor is driven away from the heat source, condenses, and flows back toward the heat source, leading to changes in porosity, permeability, temperature, saturation, and thermal conductivity of the backfill salt surrounding the waste canisters. Heat pipe formation depends on temperature, moisture availability, and mobility. In certain cases, dehydration of hydrous minerals provides sufficient extra moisture to push the system into a sustained heat pipe, where simulations neglecting this process do not.

Technical support for GEIS: radioactive waste isolation in geologic formations. Volume 8. Repository preconceptual design studies: salt

International Nuclear Information System (INIS)

1978-04-01

This volume, Volume 8 ''Repository Preconceptual Design Studies: Salt,'' is one of a 23-volume series, ''Technical Support for GEIS: Radioactive Waste Isolation in Geologic Formations,'' Y/OWI/TM-36, which supplements the ''Contribution to Draft Generic Environmental Impact Statement on Commercial Waste Management: Radioactive Waste Isolation in Geologic Formations,'' Y/OWI/TM-44. The series provides a more complete technical basis for the preconceptual designs, resource requirements, and environmental source terms associated with isolating commercial LWR wastes in underground repositories in salt, granite, shale and basalt. Wastes are considered from three fuel cycles: uranium and plutonium recycling, no recycling of spent fuel and uranium-only recycling. This document describes a preconceptual design for a nuclear waste storage facility in salt. The facility design consists of several chambers excavated deep within a geologic formation together with access shafts and supportive surface structures. The facility design provides for: receiving and unloading waste containers; lowering them down shafts to the mine level; transporting them to the proper storage area, and emplacing them in mined storage rooms. Drawings of the facility design are contained in TM-36/9, ''Drawings for Repository Preconceptual Design Studies: Salt.''

Glovebox design requirements for molten salt oxidation processing of transuranic waste

International Nuclear Information System (INIS)

Ramsey, K.B.; Acosta, S.V.; Wernly, K.D.

1998-01-01

This paper presents an overview of potential technologies for stabilization of 238 Pu-contaminated combustible waste. Molten salt oxidation (MSO) provides a method for removing greater than 99.999% of the organic matrix from combustible waste. Implementation of MSO processing at the Los Alamos National Laboratory (LANL) Plutonium Facility will eliminate the combustible matrix from 238 Pu-contaminated waste and consequently reduce the cost of TRU waste disposal operations at LANL. The glovebox design requirements for unit operations including size reduction and MSO processing will be presented

Disposal of high-level waste from nuclear power plants in Denmark. Salt dome investigations. v.5

International Nuclear Information System (INIS)

1981-01-01

The present report deals with safety evaluation as part of the investigations regarding a repository for high-level waste in a salt dome. It is volume 5 of five volumes that together constitute the final report on the Danish utilities' salt dome investigations. Two characteristics of the waste are of special importance for the safety evaluation: the encasing of the waste in steel casks with 15 cm thick walls affording protection against corrosion, protecting the surroundings against radiation, and protecting the glass cylinders from mechanical damage resulting from the pressure at the bottom of the disposal hole, and the modest generation of heat in the waste at the time of disposal resulting in a maximum temperature increase in the salt close to the waste of approx. 40 deg. C. These characteristics proved to considerably improve the safety margin with respect to unforeseen circumstances. The character of the salt dome and of the salt in the proposed disposal area offers in itself good protection against contact with the ground water outside the dome. The relatively large depth of 1200 and 2500 m of the salt surface also means that neither dome nor disposal facility will be appreciably influenced by glaciations or earthquakes. The chalk above the proposed disposal area is very tight and to retain radioactive matter effectively even in the precence of high concentrations of NaCL. The safety investigations included a number of natural processes and probable events such as the segregation of crystal water from overlooked salt minerals, faulty sealings of disposal holes, permeable fault zones in the chalk overlying the dome, the risk in connection with human penetration into the dome. These conditions will neither lead to the destruction of the waste casks or to the release of waste from the dome. Leaching of a cavern is the only situation which proved to result in a release of radioactive material to the biosphere, but the resulting doses was found to be small

Assessment of lead tellurite glass for immobilizing electrochemical salt wastes from used nuclear fuel reprocessing

Energy Technology Data Exchange (ETDEWEB)

Riley, Brian J.; Kroll, Jared O.; Peterson, Jacob A.; Pierce, David A.; Ebert, William L.; Williams, Benjamin D.; Snyder, Michelle M. V.; Frank, Steven M.; George, Jaime L.; Kruska, Karen

2017-11-01

This paper provides an overview of research evaluating the use of lead tellurite glass as a waste form for salt wastes from electrochemical reprocessing of used nuclear fuel. The efficacy of using lead tellurite glass to immobilize three different salt compositions was evaluated: a LiCl-Li2O oxide reduction salt containing fission products from oxide fuel, a LiCl-KCl eutectic salt containing fission products from metallic fuel, and SrCl2. Physical and chemical properties of glasses made with these salts were characterized with X-ray diffraction, bulk density measurements, differential thermal analysis, chemical durability tests, scanning and transmission electron microscopies, and energy-dispersive X-ray spectroscopy. These glasses were found to accommodate high salt concentrations and have high densities, but further development is needed to improve chemical durability. (C) 2017 Published by Elsevier B.V.

Applicability of molten salt oxidation to the destruction of actinide-contaminated wastes

International Nuclear Information System (INIS)

West, M.H.; Garcia, E.; Griego, W.J.; Court, D.B.; Rodriguez, L.

1992-01-01

A 1989 ban on incineration in the state of New Mexico caused cessation of actinide-contaminated cheesecloth, paper, and wood incineration within the Plutonium Facility (TA-55) at Los Alamos National Laboratory. Subsequently, plastic wipes were substituted for cheesecloth in the cleaning of glovebox interiors. However, waste minimization is not achieved by these measures since the wipes are discarded as Waste Isolation Pilot Plant certifiable wastes. After the ban was instituted, thermal decomposition of cheesecloth under argon at elevated temperature was examined and found satisfactory although scale of operation and speed were inferior to incineration. In 1991, the ban on incineration was lifted in New Mexico but Alamos has not chosen to pursue renewal of incineration at the Plutonium Facility. This paper reports that Los Alamos is looking from alternatives to incineration and thermal decomposition which are compatible with molten salt processing technology, historically a strength in actinide research at the Laboratory. Also, the technology must significantly reduce the volume of the waste upon treatment, i.e. waste minimization. Molten salt oxidation (MSO) has the promise of such a technology

Glovebox design requirements for molten salt oxidation processing of transuranic waste

Energy Technology Data Exchange (ETDEWEB)

Ramsey, K.B.; Acosta, S.V. [Los Alamos National Lab., NM (United States); Wernly, K.D. [Molten Salt Oxidation Corp., Bensalem, PA (United States)

1998-12-31

This paper presents an overview of potential technologies for stabilization of {sup 238}Pu-contaminated combustible waste. Molten salt oxidation (MSO) provides a method for removing greater than 99.999% of the organic matrix from combustible waste. Implementation of MSO processing at the Los Alamos National Laboratory (LANL) Plutonium Facility will eliminate the combustible matrix from {sup 238}Pu-contaminated waste and consequently reduce the cost of TRU waste disposal operations at LANL. The glovebox design requirements for unit operations including size reduction and MSO processing will be presented.

Recovery of plutonium and americium from chloride salt wastes by solvent extraction

International Nuclear Information System (INIS)

Reichley-Yinger, L.; Vandegrift, G.F.

1987-01-01

Plutonium and americium can be recovered from aqueous waste solutions containing a mixture of HCl and chloride salt wastes by the coupling of two solvent extraction systems: tributyl phosphate (TBP) in tetrachloroethylene (TCE) and octyl(phenyl)-N,N-diisobutylcarbamoylmethylphosphine oxide (CMPO) in TCE. In the flowsheet developed, the salt wastes are dissolved in HCl, the Pu(III) is oxidized to the IV state with NaClO 2 and recovered in the TBP-TCE cycle, and the Am is then removed from the resultant raffinate by the CMPO-TCE cycle. The consequences of the feed solution composition and extraction behavior of these species on the process flowsheet design, the Pu-product purity, and the decontamination of the aqueous raffinate from transuranic elements are discussed. 16 refs., 6 figs

Modeling internal deformation of salt structures targeted for radioactive waste disposal

International Nuclear Information System (INIS)

Chemia, Zurab

2008-01-01

This thesis uses results of systematic numerical models to argue that externally inactive salt structures, which are potential targets for radioactive waste disposal, might be internally active due to the presence of dense layers or blocks within a salt layer. The three papers that support this thesis use the Gorleben salt diapir (NW Germany), which was targeted as a future final repository for high-grade radioactive waste, as a general guideline. The first two papers present systematic studies of the parameters that control the development of a salt diapir and how it entrains a dense anhydrite layer. Results from these numerical models show that the entrainment of a dense anhydrite layer within a salt diapir depends on four parameters: sedimentation rate, viscosity of salt, perturbation width and the stratigraphic location of the dense layer. The combined effect of these four parameters, which has a direct impact on the rate of salt supply (volume/area of the salt that is supplied to the diapir with time), shape a diapir and the mode of entrainment. Salt diapirs down-built with sedimentary units of high viscosity can potentially grow with an embedded anhydrite layer and deplete their source layer (salt supply ceases). However, when salt supply decreases dramatically or ceases entirely, the entrained anhydrite layer/segments start to sink within the diapir. In inactive diapirs, sinking of the entrained anhydrite layer is inevitable and strongly depends on the rheology of the salt, which is in direct contact with the anhydrite layer. During the post-depositional stage, if the effective viscosity of salt falls below the threshold value of around 10 18 -10 19 Pa s, the mobility of anhydrite blocks might influence any repository within the diapir. However, the internal deformation of the salt diapir by the descending blocks decreases with increase in effective viscosity of salt. The results presented in this thesis suggest that it is highly likely that salt structures

Definition of the waste package environment for a repository located in salt

International Nuclear Information System (INIS)

Clark, D.E.; Bradley, D.J.

1983-01-01

The expected environmental conditions for emplaced waste packages in a salt repository are simulated in the materials testing program to evaluate performance. Synthetic brines, based on the analyses of actual brines (both intrusion and inclusion), are used for corrosion and leach testing. Elevated temperatures (to 150 0 C) and radiation fields of up to 10 3 rad/h are employed as conservative conditions to bracket expected performance and provide data for worst case scenarios. Obtaining a precise definition of the waste package environment in a salt repository and its change with time is closely tied to detailed site characterization of the candidate salt repository horizon. It is expected that field testing can augment some of the materials testing currently under way and can provide increased confidence in the predicted site-specific near-field conditions. 17 references, 5 figures, 1 table

Molten salt oxidation of mixed wastes: Separation of radioactive materials and Resource Conservation and Recovery Act (RCRA) materials

International Nuclear Information System (INIS)

Bell, J.T.; Haas, P.A.; Rudolph, J.C.

1995-01-01

The Oak Ridge National Laboratory (ORNL) is participating in a program to apply a molten salt oxidation (MSO) process to treatment of mixed (radioactive and RCRA) wastes. The salt residues from the MSO treatment will require further separations or other processing to prepare them for final disposal. A bench-scale MSO apparatus is being installed at ORNL and will be operated on real Oak Ridge wastes. The treatment concepts to be tested and demonstrated on the salt residues from real wastes are described

Analyses of SRS waste glass buried in granite in Sweden and salt in the United States

International Nuclear Information System (INIS)

Williams, J.P.; Wicks, G.G.; Clark, D.E.; Lodding, A.R.

1991-01-01

Simulated Savannah River Site (SRS) waste glass forms have been buried in the granite geology of the Stirpa mine in Sweden for two years. Analyses of glass surfaces provided a measure of the performance of the waste glasses as a function of time. Similar SRS waste glass compositions have also been buried in salt at the WIPP facility in Carlsbad, New Mexico for a similar time period. Analyses of the SRS waste glasses buried in-situ in granite will be presented and compared to the performance of these same compositions buried in salt at WIPP

Engineering study of the potential uses of salts from selective crystallization of Hanford tank wastes

International Nuclear Information System (INIS)

Hendrickson, D.W.

1996-01-01

The Clean Salt Process (CSP) is the fractional crystallization of nitrate salts from tank waste stored on the Hanford Site. This study reviews disposition options for a CSP product made from Hanford Site tank waste. These options range from public release to onsite low-level waste disposal to no action. Process, production, safety, environment, cost, schedule, and the amount of CSP material which may be used are factors considered in each option. The preferred alternative is offsite release of clean salt. Savings all be generated by excluding the material from low-level waste stabilization. Income would be received from sales of salt products. Savings and income from this alternative amount to $1,027 million, excluding the cost of CSP operations. Unless public sale of CSP products is approved, the material should be calcined. The carbonate form of the CSP could then be used as ballast in tank closure and stabilization efforts. Not including the cost of CSP operations, savings of $632 million would be realized. These savings would result from excluding the material from low-level waste stabilization and reducing purchases of chemicals for caustic recycle and stabilization and closure. Dose considerations for either alternative are favorable. No other cost-effective alternatives that were considered had the capacity to handle significant quantities of the CSP products. If CSP occurs, full-scale tank-waste stabilization could be done without building additional treatment facilities after Phase 1 (DOE 1996). Savings in capital and operating cost from this reduction in waste stabilization would be in addition to the other gains described

Guidelines for the Development of Comprehensive Care Centers for Congenital Adrenal Hyperplasia: Guidance from the CARES Foundation Initiative

Directory of Open Access Journals (Sweden)

Rink RichardC

2010-11-01

Full Text Available Patients with rare and complex diseases such as congenital adrenal hyperplasia (CAH often receive fragmented and inadequate care unless efforts are coordinated among providers. Translating the concepts of the medical home and comprehensive health care for individuals with CAH offers many benefits for the affected individuals and their families. This manuscript represents the recommendations of a 1.5 day meeting held in September 2009 to discuss the ideal goals for comprehensive care centers for newborns, infants, children, adolescents, and adults with CAH. Participants included pediatric endocrinologists, internal medicine and reproductive endocrinologists, pediatric urologists, pediatric surgeons, psychologists, and pediatric endocrine nurse educators. One unique aspect of this meeting was the active participation of individuals personally affected by CAH as patients or parents of patients. Representatives of Health Research and Services Administration (HRSA, New York-Mid-Atlantic Consortium for Genetics and Newborn Screening Services (NYMAC, and National Newborn Screening and Genetics Resource Center (NNSGRC also participated. Thus, this document should serve as a "roadmap" for the development phases of comprehensive care centers (CCC for individuals and families affected by CAH.

Forecasting the space-time stability of radioactive waste isolation in salt formations

International Nuclear Information System (INIS)

Anderson, E.B.; Karelin, A.I.; Krivokhatsiy, A.S.; Savonenkov, V.G.

1992-01-01

The possibilities to use salt formations for radioactive waste isolation are realized by creating shaft-type underground repositories in these rocks in Germany and the USA. The burial safety of low- and intermediate-level wastes for several hundred years have been substantiated for the sites chosen. Specialists of different countries presented positive properties of rock salt as a medium for isolation of radionuclides. A rich experience in building subsurface structures for different purposes in salts is accumulated in our country. Detailed investigations of salt formation have shown that far from all the saliferous areas and structures may be used for constructing burial sites. One of the reasons for this limitation is a sharp difference of individual deposits by their compositions, structures, the character of deposition and the conditions of formation. The geological criteria of safety acquire special significance in connection with the necessity to isolate radionuclides having the half-loves more than 1000 years. The time intervals required for stable isolation make up millions of years and cover great cycles of the evolution of the Earth surface and biosphere

Release consequence analysis for a hypothetical geologic radioactive waste repository in salt

International Nuclear Information System (INIS)

1979-08-01

One subtask conducted under the INFCE program is to evaluate and compare the health and safety impacts of different fuel cycles in which all radioactive wastes (except those from mining and milling) are placed in a geologic repository in salt. To achieve this objective, INFCE Working Group 7 examined the radiologic dose to humans from geologic repositories containing waste arisings as defined for seven reference fuel cycles. This report examines the release consequences for a generic waste repository in bedded salt. The top of the salt formation and the top of the repository are assumed to be 250 and 600 m, respectively, below the surface. The hydrogeologic structure above the salt consists of two aquifers and two aquitards. The aquifers connect to a river 6.2 km from the repository. The regional gradient to the river is 1 m/km in all aquifers. Hydrologic, transport, and dose models were used to model two release scenarios for each fuel cycle, one without a major disturbance and one in which a major geologic perturbation breached the repository immediately after it was sealed. The purpose of the modeling was to predict the rate of transport of radioactive contaminants from the repository through the geosphere to the biosphere, and to determine the potential dose to humans. Of the many radionuclides in the waste, only 129 I and 226 Ra arrived at the river in sufficient concentrations for a measurable dose calculation. Radionuclide concentrations in the ground water pose no threat to man because the ground water is a concentrated brine and it is diluted by a factor of 10 6 to 10 7 upon entering the river

Salt creep design consideration for underground nuclear waste storage

International Nuclear Information System (INIS)

Li, W.T.; Wu, C.L.; Antonas, N.J.

1983-01-01

This paper summarizes the creep consideration in the design of nuclear waste storage facilities in salt, describes the non-linear analysis method for evaluating the design adequacy, and presents computational results for the current storage design. The application of rock mechanics instrumentation to assure the appropriateness of the design is discussed. It also describes the design evolution of such a facility, starting from the conceptual design, through the preliminary design, to the detailed design stage. The empirical design method, laboratory tests and numerical analyses, and the underground in situ tests have been incorporated in the design process to assure the stability of the underground openings, retrievability of waste during the operation phase and encapsulation of waste after decommissioning

Prevalence of Nonclassic Congenital Adrenal Hyperplasia in Turkish Children Presenting with Premature Pubarche, Hirsutism, or Oligomenorrhoea

Directory of Open Access Journals (Sweden)

Cigdem Binay

2014-01-01

Full Text Available Background. Nonclassic congenital adrenal hyperplasia (NCAH, caused by mutations in the gene encoding 21-hydroxylase, is a common autosomal recessive disorder. In the present work, our aim was to determine the prevalence of NCAH presenting as premature pubarche (PP, hirsutism, or polycystic ovarian syndrome (PCOS and to evaluate the molecular spectrum of CYP21A2 mutations in NCAH patients. Methods. A total of 126 patients (122 females, 4 males with PP, hirsutism, or PCOS were included in the present study. All patients underwent an ACTH stimulation test. NCAH was considered to be present when the stimulated 17-hydroxyprogesterone plasma level was >10 ng/mL. Results. Seventy-one of the 126 patients (56% presented with PP, 29 (23% with PCOS, and 26 (21% with hirsutism. Six patients (4,7% were diagnosed with NCAH based on mutational analysis. Four different mutations (Q318X, P30L, V281L, and P453S were found in six NCAH patients. One patient with NCAH was a compound heterozygote for this mutation, and five were heterozygous. Conclusion. NCAH should be considered as a differential diagnosis in patients presenting with PP, hirsutism, and PCOS, especially in countries in which consanguineous marriages are prevalent.

Performance Assessment of a Generic Repository in Bedded Salt for DOE-Managed Nuclear Waste

Science.gov (United States)

Stein, E. R.; Sevougian, S. D.; Hammond, G. E.; Frederick, J. M.; Mariner, P. E.

2016-12-01

A mined repository in salt is one of the concepts under consideration for disposal of DOE-managed defense-related spent nuclear fuel (SNF) and high level waste (HLW). Bedded salt is a favorable medium for disposal of nuclear waste due to its low permeability, high thermal conductivity, and ability to self-heal. Sandia's Generic Disposal System Analysis framework is used to assess the ability of a generic repository in bedded salt to isolate radionuclides from the biosphere. The performance assessment considers multiple waste types of varying thermal load and radionuclide inventory, the engineered barrier system comprising the waste packages, backfill, and emplacement drifts, and the natural barrier system formed by a bedded salt deposit and the overlying sedimentary sequence (including an aquifer). The model simulates disposal of nearly the entire inventory of DOE-managed, defense-related SNF (excluding Naval SNF) and HLW in a half-symmetry domain containing approximately 6 million grid cells. Grid refinement captures the detail of 25,200 individual waste packages in 180 disposal panels, associated access halls, and 4 shafts connecting the land surface to the repository. Equations describing coupled heat and fluid flow and reactive transport are solved numerically with PFLOTRAN, a massively parallel flow and transport code. Simulated processes include heat conduction and convection, waste package failure, waste form dissolution, radioactive decay and ingrowth, sorption, solubility limits, advection, dispersion, and diffusion. Simulations are run to 1 million years, and radionuclide concentrations are observed within an aquifer at a point approximately 4 kilometers downgradient of the repository. The software package DAKOTA is used to sample likely ranges of input parameters including waste form dissolution rates and properties of engineered and natural materials in order to quantify uncertainty in predicted concentrations and sensitivity to input parameters. Sandia

Computer simulation of an internally pressurized radioactive waste disposal room in a bedded salt formation

International Nuclear Information System (INIS)

Brown, W.T.; Weatherby, J.R.

1991-01-01

The Waste Isolation Pilot Plant (WIPP) near Carlsbad, New Mexico was created by the U.S. Department of Energy as an underground research and development facility to demonstrate the safe storage of transuranic waste generated from defense activities. This facility consists of storage rooms mined from a bedded salt formation at a depth of about 650 meters. Each room will accommodate about 6800 55-gallon drums filled with waste. After waste containers are emplaced, the storage rooms are to be backfilled with mined salt or other backfill materials. As time passes, reconsolidation of this backfill will reduce the hydraulic conductivity of the room. However, gases produced by decomposition and corrosion of waste and waste containers may cause a slow build-up of pressure which can retard consolidation of the waste and backfilled salt. The authors have developed a finite-element model of an idealized disposal room which is assumed to be perfectly sealed. The assumption that no gas escapes from the disposal room is a highly idealized and extreme condition which does not account for leakage paths, such as interbeds, that exist in the surrounding salt formation. This model has been used in a parametric study to determine how reconsolidation is influenced by various assumed gas generation rates and total amounts of gas generated. Results show that reductions in the gas generation, relative to the baseline case, can increase the degree of consolidation and reduce the peak gas pressure in disposal rooms. Even higher degrees of reconsolidation can be achieved by reducing both amounts and rates of gas generation. 8 refs., 4 figs., 1 tab

Possible salt mine and brined cavity sites for radioactive waste disposal in the northeastern southern peninsula of Michigan

International Nuclear Information System (INIS)

Landes, K.K.; Bourne, H.L.

1976-01-01

A reconnaissance report on the possibilities for disposal of radioactive waste covers Michigan only, and is more detailed than an earlier one involving the northeastern states. Revised ''ground rules'' for pinpointing both mine and dissolved salt cavern sites for waste disposal include environmental, geologic, and economic factors. The Michigan basin is a structural bowl of Paleozoic sediments resting on downwarped Precambrian rocks. The center of the bowl is in Clare and Gladwin Counties, a short distance north of the middle of the Southern Peninsula. The strata dip toward this central area, and some stratigraphic sequences, including especially the salt-containing Silurian section, increase considerably in thickness in that direction. Lesser amounts of salt are also present in the north central part of the Lower Peninsula. Michigan has been an oil and gas producing state since 1925 and widespread exploration has had two effects on the selection of waste disposal sites: (1) large areas are leased for oil and gas; and (2) the borehole concentrations, whether producing wells, dry holes, or industrial brine wells that penetrated the salt section, should be avoided. Two types of nuclear waste, low level and high level, can be stored in man-made openings in salt beds. The storage facilities are created by (1) the development of salt mines where the depths are less than 3000 ft, and (2) cavities produced by pumping water into a salt bed, and bringing brine back out. The high level waste disposal must be confined to mines of limited depth, but the low level wastes can be accommodated in brine cavities at any depth. Seven potential prospects have been investigated and are described in detail

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.

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.

Nuclear waste repository simulation experiments. Asse salt mine: Annual report 1984

International Nuclear Information System (INIS)

Rothfuchs, T.; Feddersen, H.K.; Schwarzianeck, P.; Staupendahl, G.; Coyle, A.J.; Kalia, H.; Eckert, J.

1985-01-01

This is the Second Annual Report (1984) which describes experiments simulating a nuclear waste repository at the 800 meter-level of the Asse Salt Mine in the Federal Republic of Germany. The report describes the Asse Salt Mine, the test equipment, and the pretest properties of the salt in the mine and in the vicinity of the test area. Also included are test data for the first sixteen months of operation on the following: brine migration rates, thermal mechanical behavior of the salt (including room closure, stress readings and thermal profiles) and borehole gas pressures. In addition to field data laboratory analyses of results are also included in this report. The duration of the experiment will be two years, ending in December 1985. (orig.)

Hydrothermal preparation of zeolite Li-A and ion exchange properties of Cs and Sr in salt waste

International Nuclear Information System (INIS)

Lee, S. H.; Kim, J. G.; Lee, J. H.; Kim, J. H.

2005-01-01

An advanced spent fuel management process that were based on Li reduction of the oxide spent fuel to a metallic form will generate a LiCl waste. Zeolite A has been reported as a promising immobilization medium for waste salt with CsCl and SrCl 2 . However, Sodium is accumulated as an ionic form (Na + -ion) in molten salt during ion exchange step between Na + -ion in zeolite A and Li + -ion in the molten salt. Therefore, zeolite Na-A need to be replaced by the Li-type zeolite for recycling the salt waste by removing the Cs and Sr ions. In this study, the hydrothermal preparation of zeolite Li-A was performed in 350ml pressure vessel by P. Norby method. The preparation characteristics of zeolite Li-A was investigated. And the ion exchange properties of Cs and Sr in molten LiCl salt were investigated under the condition of 923K using zeolite 4A and prepared zeolite Li-A

Corrosion aspects of high-level waste disposal in salt domes

International Nuclear Information System (INIS)

Roerbo, K.

1979-12-01

In the ELSAM/ELKRAT waste management project it is planned that the high-level waste is glassified, encapsuled in canisters and finally deposited in a deep hole drilled in a salt dome. In the present report corrosion aspects of the canisters after deposition are discussed. The chemical environment will probably be a limited amount of brine coming from brine inclusions in the surrounding salt and moving up against the temperature gradient, the temperature at the canister surface being in the range of 100-150degC. The possible types of corrosion and the expected corrosion rates for a number of potential canister materials (mild steel, austenitic and ferritic stainless steels, Ni-base alloys, copper, titanium and a few combinations of materials) are discussed. Mild steel (possibly combined with an inner layer of copper or titanium) might possibly be an appropriate choice of material for the canister. (author)

Radiological consequences of a human intrusion in a nuclear waste repository in a salt formation

International Nuclear Information System (INIS)

Jacquier, P.; Raimbault, P.

1989-07-01

The assessment of the consequences of human intrusion scenarios for a repository is very important for salt formations, since this material has an undeniable economic interest. In this work, the scenario considers the solution mining of salt for human consumption: salt is extracted from a cavern; by leaching, this cavern enlarges and uncovers the waste, which falls down into the sump. It was assumed that the intrusion takes place either 500 years or 2500 years after the closing of the repository. High-level vitrified waste or alpha cemented waste were considered. This paper displays the assumptions made and, using a simplified modelling of the phenomena, the estimation of the radiological consequences due to ingestion of contamined sals. A sensitivity/uncertainty analysis is presented which emphasizes several fields where experimental studies have to be pursued or launched [fr

High-Level Waste Salt Disposition Systems Engineering Team Final Report, Volumes I, II, and III

International Nuclear Information System (INIS)

Piccolo, S.F.

1999-01-01

This report describes the process used and results obtained by the High Level Waste Salt Disposition Systems Engineering Team to select a primary and backup alternative salt disposition method for the Savannah River Site

Analysis of the geological stability of a hypothetical radioactive waste repository in a bedded salt formation

International Nuclear Information System (INIS)

Tierney, M.S.; Lusso, F.; Shaw, H.R.

1978-01-01

This document reports on the development of mathematical models used in preliminary studies of the long-term safety of radioactive wastes deeply buried in bedded salt formations. Two analytical approaches to estimating the geological stability of a waste repository in bedded salt are described: (a) use of probabilistic models to estimate the a priori likelihoods of release of radionuclides from the repository through certain idealized natural and anthropogenic causes, and (b) a numerical simulation of certain feedback effects of emplacement of waste materials upon ground-water access to the repository's host rocks. These models are applied to an idealized waste repository for the sake of illustration

Thermal properties of fly ash substituted slag cement waste forms for disposal of Savannah River Plant salt waste

International Nuclear Information System (INIS)

Roy, D.M.; Kaushal, S.; Licastro, P.H.; Langton, C.A.

1985-01-01

Waste processing at the Savannah River Plant will involve reconstitution of the salts (NaNO 3 , NaNO 2 , NaOH, etc.) into a concentrated solution (32 weight percent salts) followed by solidification in a cement-based waste form for burial. The stability and mechanical durability of such a 'saltstone monolith' will depend largely on the temperature reached due to heat of hydration and the thermal properties of the waste form. Fly ash has been used as an inexpensive constituent and to moderate the hydration and setting processes so as to avoid reaching prohibitively high temperatures which could cause thermal stresses. Both high-calcium and low-calcium fly ashes have been studied for this purpose. Other constituents of these mixes include granulated blast furnace slag and finely crushed limestone. Adiabatic temperature increase and thermal conductivity of these mixes have been studied and related x-ray diffraction and scanning electron microscopy studies carried out to understand the hydration process

Subsurface geology of a potential waste emplacement site, Salt Valley Anticline, Grand County, Utah

Science.gov (United States)

Hite, R.J.

1977-01-01

The Salt Valley anticline, which is located about 32 km northeast of Moab, Utah, is perhaps one of the most favorable waste emplacement sites in the Paradox basin. The site, which includes about 7.8 km 2, is highly accessible and is adjacent to a railroad. The anticline is one of a series of northwest-trending salt anticlines lying along the northeast edge of the Paradox basin. These anticlines are cored by evaporites of the Paradox Member of the Hermosa Formation of Middle Pennsylvanian age. The central core of the Salt Valley anticline forms a ridgelike mass of evaporites that has an estimated amplitude of 3,600 m. The evaporite core consists of about 87 percent halite rock, which includes some potash deposits; the remainder is black shale, silty dolomite, and anhydrite. The latter three lithologies are referred to as 'marker beds.' Using geophysical logs from drill holes on the anticline, it is possible to demonstrate that the marker beds are complexly folded and faulted. Available data concerning the geothermal gradient and heatflow at the site indicate that heat from emplaced wastes should be rapidly dissipated. Potentially exploitable resources of potash and petroleum are present at Salt Valley. Development of these resources may conflict with use of the site for waste emplacement.

Subsurface geology of a potential waste emplacement site, Salt Valley Anticline, Grand County, Utah

International Nuclear Information System (INIS)

Hite, R.J.

1977-01-01

The Salt Valley anticline, which is located about 32 km northeast of Moab, Utah, is perhaps one of the most favorable waste emplacement sites in the Paradox basin. The site, which includes about 7.8 km 2 , is highly accessible and is adjacent to a railroad. The anticline is one of a series of northwest-trending salt antilcines lying along the northeast edge of the Paradox basin. These anticlines are cored by evaporites of the Paradox Member of the Hermosa Formation of Middle Pennsylvanian age. The central core of the Salt Valley anticline forms a ridgelike mass of evaporites that has an estimated amplitude of 3,600 m. The evaporite core consists of about 87 percent halite rock, which includes some potash deposits; the remainder is black shale, silty dolomite, and anhydrite. The latter three lithologies are referred to as ''marker beds.'' Using geophysical logs from drill holes on the anticline, it is possible to demonstrate that the marker beds are complexly folded and faulted. Available data concerning the geothermal gradient and heatflow at the site indicate that heat from emplaced wastes should be rapidly dissipated. Potentially exploitable resources of potash and petroleum are present at Salt Valley. Development of these resources may conflict with use of the site for waste emplacement

[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.

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.

Principles and clinical applications of liquid chromatography - tandem mass spectrometry for the determination of adrenal and gonadal steroid hormones.

Science.gov (United States)

Kulle, A E; Welzel, M; Holterhus, P-M; Riepe, F G

2011-10-01

Liquid-chromatography - tandem mass spectrometry (LC-MS/MS) is becoming the method of choice for clinical steroid analysis. In most instances, it has the advantage of higher sensitivity, better reproducibility and greater specificity than commercial immunoassay techniques. The method requires only minimal sample preparation and a small sample volume. Furthermore, it has the potential to analyze multiple steroids simultaneously. Modern instruments guarantee high throughput, allowing an affordable price for the individual assay. All this makes LC-MS/MS an attractive method for use in a clinical setting. Reliable reference ranges for the detected analytes are the pre-requisite for their clinical use. If these are available, LC-MS/MS can find application in congenital disorders of steroid metabolism, such as congenital adrenal hyperplasia, disorders of sex development and disorders of salt homeostasis, as well as in acquired disorders of steroid metabolism, such as primary aldosteronism, Cushing's disease, Addison's disease, and hyperandrogenemia, as well as in psychiatric disease states such as depression or anxiety disorders. The principles of LC-MS/MS for steroid measurement, the pros and cons of LC-MS/MS compared with conventional immunoassays and the possible applications in clinical routine, with a special focus on pediatric endocrinology needs, are discussed here.

Equipment evaluation for low density polyethylene encapsulated nitrate salt waste at the Rocky Flats Plant

International Nuclear Information System (INIS)

Yamada, W.I.; Faucette, A.M.; Jantzen, R.C.; Logsdon, B.W.; Oldham, J.H.; Saiki, D.M.; Yudnich, R.J.

1993-01-01

Mixed wastes at the Rocky Flats Plant (RFP) are subject to regulation by the Resource Conservation and Recovery Act (RCRA). Polymer solidification is being developed as a final treatment technology for several of these mixed wastes, including nitrate salts. Encapsulation nitrate salts with low density polyethylene (LDPE) has been the preliminary focus of the RFP polymer solidification effort. Literature reviews, industry surveys, and lab-scale and pilot-scale tests have been conducted to evaluate several options for encapsulating nitrate salts with LDPE. Most of the effort has focused on identifying compatible drying and extrusion technologies. Other processing options, specifically meltration and non-heated compounding machines, were also investigated. The best approach appears to be pretreatment of the nitrate salt waste brine in either a vertical or horizontal thin film evaporator followed by compounding of the dried waste with LDPE in an intermeshing, co-rotating, twin-screw extruder. Additional pilot-scale tests planned for the fall of 1993 should further support this recommendation. Preliminary evaluation work indicates that meltration is not possible at atmospheric pressure with the LDPE (Chevron PE-1409) provided by RFP. However, meltration should be possible at atmospheric pressure using another LDPE formulation with altered physical and rheological properties: Lower molecular weight and lower viscosity (Epoline C-15). Contract modifications are now in process to allow a follow-on pilot scale demonstration. Questions regarding changed safety and physical properties of the resultant LDPE waste form due to use of the Epoline C-15 will be addressed. No additional work with non-heated mixer compounder machines is planned at this time

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

Directory of Open Access Journals (Sweden)

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.

Solubility and speciation of actinides in salt solutions and migration experiments of intermediate level waste in salt formations

International Nuclear Information System (INIS)

1986-01-01

A comprehensive study into the solubility of the actinides americium and plutonium in concentrated salt solutions, the release of radionuclides from various forms of conditioned ILW and the migration behaviour of these nuclides through geological material specific to the Gorleben site in Lower Saxony is described. A detailed investigation into the characterization of four highly concentrated salt solutions in terms of their pH, Eh, inorganic carbon contents and their densities is given and a series of experiments investigating the solubility of standard americium(III) and plutonium(IV) hydroxides in these solutions is described. Transuranic mobility studies for solutions derived from the standard hydroxides through salt and sand have shown the presence of at least two types of species present of widely differing mobility; one migrating with approximately the same velocity as the solvent front and the other strongly retarded. Actinide mobility data are presented and discussed for leachates derived from the simulated ILW in cement and data are also presented for the migration of the fission products in leachates derived from real waste solidified in cement and bitumen. Relatively high plutonium mobilities were observed in the case of the former and in the case of the real waste leachates, cesium was found to be the least retarded. The sorption of ruthenium was found to be largely associated with the insoluble residues of the natural rock salt rather than the halite itself. (orig./RB)

Novel waste printed circuit board recycling process with molten salt.

Science.gov (United States)

Riedewald, Frank; Sousa-Gallagher, Maria

2015-01-01

The objective of the method was to prove the concept of a novel waste PCBs recycling process which uses inert, stable molten salts as the direct heat transfer fluid and, simultaneously, uses this molten salt to separate the metal products in either liquid (solder, zinc, tin, lead, etc.) or solid (copper, gold, steel, palladium, etc.) form at the operating temperatures of 450-470 °C. The PCB recovery reactor is essentially a U-shaped reactor with the molten salt providing a continuous fluid, allowing molten salt access from different depths for metal recovery. A laboratory scale batch reactor was constructed using 316L as suitable construction material. For safety reasons, the inert, stable LiCl-KCl molten salts were used as direct heat transfer fluid. Recovered materials were washed with hot water to remove residual salt before metal recovery assessment. The impact of this work was to show metal separation using molten salts in one single unit, by using this novel reactor methodology. •The reactor is a U-shaped reactor filled with a continuous liquid with a sloped bottom representing a novel reactor concept.•This method uses large PCB pieces instead of shredded PCBs as the reactor volume is 2.2 L.•The treated PCBs can be removed via leg B while the process is on-going.

Novel waste printed circuit board recycling process with molten salt

Science.gov (United States)

Riedewald, Frank; Sousa-Gallagher, Maria

2015-01-01

The objective of the method was to prove the concept of a novel waste PCBs recycling process which uses inert, stable molten salts as the direct heat transfer fluid and, simultaneously, uses this molten salt to separate the metal products in either liquid (solder, zinc, tin, lead, etc.) or solid (copper, gold, steel, palladium, etc.) form at the operating temperatures of 450–470 °C. The PCB recovery reactor is essentially a U-shaped reactor with the molten salt providing a continuous fluid, allowing molten salt access from different depths for metal recovery. A laboratory scale batch reactor was constructed using 316L as suitable construction material. For safety reasons, the inert, stable LiCl–KCl molten salts were used as direct heat transfer fluid. Recovered materials were washed with hot water to remove residual salt before metal recovery assessment. The impact of this work was to show metal separation using molten salts in one single unit, by using this novel reactor methodology. • The reactor is a U-shaped reactor filled with a continuous liquid with a sloped bottom representing a novel reactor concept. • This method uses large PCB pieces instead of shredded PCBs as the reactor volume is 2.2 L. • The treated PCBs can be removed via leg B while the process is on-going. PMID:26150977

NRC Monitoring of Salt Waste Disposal at the Savannah River Site - 13147

Energy Technology Data Exchange (ETDEWEB)

Pinkston, Karen E.; Ridge, A. Christianne; Alexander, George W.; Barr, Cynthia S.; Devaser, Nishka J.; Felsher, Harry D. [U.S. Nuclear Regulatory Commission (United States)

2013-07-01

As part of monitoring required under Section 3116 of the Ronald W. Reagan National Defense Authorization Act for Fiscal Year 2005 (NDAA), the NRC staff reviewed an updated DOE performance assessment (PA) for salt waste disposal at the Saltstone Disposal Facility (SDF). The NRC staff concluded that it has reasonable assurance that waste disposal at the SDF meets the 10 CFR 61 performance objectives for protection of individuals against intrusion (chap.61.42), protection of individuals during operations (chap.61.43), and site stability (chap.61.44). However, based on its evaluation of DOE's results and independent sensitivity analyses conducted with DOE's models, the NRC staff concluded that it did not have reasonable assurance that DOE's disposal activities at the SDF meet the performance objective for protection of the general population from releases of radioactivity (chap.61.41) evaluated at a dose limit of 0.25 mSv/yr (25 mrem/yr) total effective dose equivalent (TEDE). NRC staff also concluded that the potential dose to a member of the public is expected to be limited (i.e., is expected to be similar to or less than the public dose limit in chap.20.1301 of 1 mSv/yr [100 mrem/yr] TEDE) and is expected to occur many years after site closure. The NRC staff used risk insights gained from review of the SDF PA, its experience monitoring DOE disposal actions at the SDF over the last 5 years, as well as independent analysis and modeling to identify factors that are important to assessing whether DOE's disposal actions meet the performance objectives. Many of these factors are similar to factors identified in the NRC staff's 2005 review of salt waste disposal at the SDF. Key areas of interest continue to be waste form and disposal unit degradation, the effectiveness of infiltration and erosion controls, and estimation of the radiological inventory. Based on these factors, NRC is revising its plan for monitoring salt waste disposal at the SDF in

NRC Monitoring of Salt Waste Disposal at the Savannah River Site - 13147

International Nuclear Information System (INIS)

Pinkston, Karen E.; Ridge, A. Christianne; Alexander, George W.; Barr, Cynthia S.; Devaser, Nishka J.; Felsher, Harry D.

2013-01-01

As part of monitoring required under Section 3116 of the Ronald W. Reagan National Defense Authorization Act for Fiscal Year 2005 (NDAA), the NRC staff reviewed an updated DOE performance assessment (PA) for salt waste disposal at the Saltstone Disposal Facility (SDF). The NRC staff concluded that it has reasonable assurance that waste disposal at the SDF meets the 10 CFR 61 performance objectives for protection of individuals against intrusion (chap.61.42), protection of individuals during operations (chap.61.43), and site stability (chap.61.44). However, based on its evaluation of DOE's results and independent sensitivity analyses conducted with DOE's models, the NRC staff concluded that it did not have reasonable assurance that DOE's disposal activities at the SDF meet the performance objective for protection of the general population from releases of radioactivity (chap.61.41) evaluated at a dose limit of 0.25 mSv/yr (25 mrem/yr) total effective dose equivalent (TEDE). NRC staff also concluded that the potential dose to a member of the public is expected to be limited (i.e., is expected to be similar to or less than the public dose limit in chap.20.1301 of 1 mSv/yr [100 mrem/yr] TEDE) and is expected to occur many years after site closure. The NRC staff used risk insights gained from review of the SDF PA, its experience monitoring DOE disposal actions at the SDF over the last 5 years, as well as independent analysis and modeling to identify factors that are important to assessing whether DOE's disposal actions meet the performance objectives. Many of these factors are similar to factors identified in the NRC staff's 2005 review of salt waste disposal at the SDF. Key areas of interest continue to be waste form and disposal unit degradation, the effectiveness of infiltration and erosion controls, and estimation of the radiological inventory. Based on these factors, NRC is revising its plan for monitoring salt waste disposal at the SDF in coordination with South

Growth hormone therapy alone or in combination with gonadotropin-releasing hormone analog therapy to improve the height deficit in children with congenital adrenal hyperplasia.

Science.gov (United States)

Quintos, J B; Vogiatzi, M G; Harbison, M D; New, M I

2001-04-01

Short stature in the adult patient with congenital adrenal hyperplasia (CAH) is commonly seen, even among patients in excellent adrenal control during childhood and puberty. In this study we examine the effect of GH therapy on height prediction in children with both CAH and compromised height prediction. Leuprolide acetate, a GnRH analog (GnRHa), was given to patients with evidence of early puberty. GH (n = 12) or the combination of GH and GnRHa (n = 8) was administered to 20 patients with CAH while they continued therapy with glucocorticoids. Each patient in the treatment group was matched according to age, sex, bone age, puberty, and type of CAH with another CAH patient treated only with glucocorticoid replacement. The match was made at the start of GH treatment. Of the 20 patients, 12 have completed 2 yr of therapy. After 1 yr of GH or combination GH and GnRHa therapy, the mean growth rate increased from 5 +/- 1.9 to 7.8 +/- 1.6 cm/yr vs. 5.4 +/- 1.7 to 5 +/- 2 cm/yr in the group not receiving GH (P growth rate was 6 +/- 1.6 vs. 4.2 +/- 2.1 cm/yr in the group not receiving GH (P growth rate and height prediction and a reduction in height deficit for bone age.

Radioactive waste isolation in salt: peer review of the Office of Nuclear Waste Isolation's report on Functional Design Criteria for a Repository for High-Level Radioactive Waste

International Nuclear Information System (INIS)

Hambley, D.F.; Russell, J.E.; Busch, J.S.; Harrison, W.; Edgar, D.E.; Tisue, M.W.

1984-08-01

This report summarizes Argonne's review of the Office of Nuclear Waste Isolation's (ONWI's) draft report entitled Functional Design Criteria for High-Level Nuclear Waste Repository in Salt, dated January 23, 1984. Recommendations are given for improving the ONWI draft report

Reconsolidation of salt as applied to permanent seals for the Waste Isolation Pilot Plant

International Nuclear Information System (INIS)

Hansen, F.D.; Callahan, G.D.; Van Sembeek, L.L.

1993-01-01

Reconsolidated salt is a fundamental component of the permanent seals for the Waste Isolation Pilot Plant. As regulations are currently understood and seal concepts envisioned, emplaced salt is the sole long-term seal component designed to prevent the shafts from becoming preferred pathways for rating gases or liquids. Studies under way in support of the sealing function of emplaced salt include laboratory testing of crushed salt small-scale in situ tests, constitutive modeling of crushed salt, calculations of the opening responses during operation and closure, and design practicalities including emplacement techniques. This paper briefly summarizes aspects of these efforts and key areas of future work

Implications of thermophysical properties in geoscientific investigations for the disposal of nuclear waste in a salt dome

International Nuclear Information System (INIS)

Kopietz, J.

1984-01-01

Examples from laboratory and in-situ experiments on the thermomechanical behavior of rock salt are used to discuss the implications of thermophysical properties for disposal of nuclear waste in a salt dome. The implications of thermophysical properties are also illustrated by a brief review of geothermal investigations made within the scope of geological and hydrogeological exploration of the Gorleben salt dome in northern Germany. High-resolution temperature measurements performed in shallow and deep boreholes drilled for the exploration of the Gorleben salt dome, together with thermal conductivity measurements on representative core samples from these boreholes, are contributing to a determination of groundwater flow in the covering layers of the salt dome and to the identification of zones of impurity (eg carnallitite layers) within the salt structure. Data from these experiments are used for setting up numerical models for heat propagation around a prospective waste repository in the Gorleben salt dome. Long-term creep experiments on samples of rock salt at up to 400 deg C are used to derive constitutive relations on the creep behavior of salt. In-situ heating experiments are being conducted in the Asse salt mine to determine the effect of a heat source on the integrity of the surrounding salt rock. (author)

Defense Waste Processing Facility (DWPF), Modular CSSX Unit (CSSX), and Waste Transfer Line System of Salt Processing Program (U)

International Nuclear Information System (INIS)

CHANG, ROBERT

2006-01-01

All of the waste streams from ARP, MCU, and SWPF processes will be sent to DWPF for vitrification. The impact these new waste streams will have on DWPF's ability to meet its canister production goal and its ability to support the Salt Processing Program (ARP, MCU, and SWPF) throughput needed to be evaluated. DWPF Engineering and Operations requested OBU Systems Engineering to evaluate DWPF operations and determine how the process could be optimized. The ultimate goal will be to evaluate all of the Liquid Radioactive Waste (LRW) System by developing process modules to cover all facilities/projects which are relevant to the LRW Program and to link the modules together to: (1) study the interfaces issues, (2) identify bottlenecks, and (3) determine the most cost effective way to eliminate them. The results from the evaluation can be used to assist DWPF in identifying improvement opportunities, to assist CBU in LRW strategic planning/tank space management, and to determine the project completion date for the Salt Processing Program

Permian salt dissolution, alkaline lake basins, and nuclear-waste storage, Southern High Plains, Texas and New Mexico

International Nuclear Information System (INIS)

Reeves, C.C. Jr.; Temple, J.M.

1986-01-01

Areas of Permian salt dissolution associated with 15 large alkaline lake basins on and adjacent to the Southern High Plains of west Texas and eastern New Mexico suggest formation of the basins by collapse of strata over the dissolution cavities. However, data from 6 other alkaline basins reveal no evidence of underlying salt dissolution. Thus, whether the basins were initiated by subsidence over the salt dissolution areas or whether the salt dissolution was caused by infiltration of overlying lake water is conjectural. However, the fact that the lacustrine fill in Mound Lake greatly exceeds the amount of salt dissolution and subsidence of overlying beds indicates that at least Mound Lake basin was antecedent to the salt dissolution. The association of topography, structure, and dissolution in areas well removed from zones of shallow burial emphasizes the susceptibility of Permian salt-bed dissolution throughout the west Texas-eastern New Mexico area. Such evidence, combined with previous studies documenting salt-bed dissolution in areas surrounding a proposed high-level nuclear-waste repository site in Deaf Smith County, Texas, leads to serious questions about the rationale of using salt beds for nuclear-waste storage

Salt Repository Project: Waste Package Program (WPP) modeling activiteis: FY 1984 annual report

International Nuclear Information System (INIS)

Kuhn, W.L.; Simonson, S.A.; Pulsipher, B.A.

1987-03-01

The Pacific Northwest Laboratory (PNL) is supporting the US Department of Energy's (DOE) Salt Repository Project (SRP) through its Waste Package Program (WPP). During FY 1984, the WPP continued its program of waste package component development and interactions testing and application of the resulting data base to develop predictive models describing waste package degradation and radionuclide release. Within the WPP, the Modeling Task (Task 04 during FY 1984) was conducted to interpret the tests in such a way that scientifically defensible models can be developed for use in qualification of the waste package

[Clinical management of adrenal incidentalomas: results of a survey].

Science.gov (United States)

Moreno-Fernández, Jesús; García-Manzanares, Alvaro; Sánchez-Covisa, Miguel Aguirre; García, E Inés Rosa Gómez

2009-12-01

Incidentalomas are clinically silent adrenal masses that are discovered incidentally during diagnostic testing for clinical conditions unrelated to suspicion of adrenal disease. Several decision algorithms are used in the management of adrenal masses. We evaluated the routine use of these algorithms through a clinical activity questionnaire. The questionnaire included data on the work center, initial hormonal and radiological study, imaging and hormonal tests performed to complete the study, surgical indications and clinical follow-up. Thirty-three endocrinologists (79%) attending the annual congress of the Castilla-La Mancha Society of Endocrinology, Nutrition and Diabetes completed the questionnaire. Forty-six percent considered tumoral size to be the most important factor suggesting malignancy in the initial evaluation of adrenal incidentalomas, the limit being 4 cm for 78% of the endocrinologists. Imaging study was completed by magnetic resonance imaging by 39%. All the physicians always performed screening for hypercortisolism and pheochromocytoma. Other assessments always conducted in all incidentalomas included hyperaldosteronism (76%), sex hormone-producing tumor (51%) and congenital adrenal hyperplasia (30%). Seventy-nine percent of respondents began to refer incidentalomas larger than 4 cm for surgical treatment, and 46% referred all tumors larger than 6 cm for surgical treatment. With regard to hormonal function, patients with pheochromocytoma, Cushing's syndrome, hyperaldosteronism with poorly controlled blood pressure or sex hormoneproducing tumors were more frequently referred for surgery. Seventy-six percent of endocrinologists performed clinical follow-up in adrenal incidentalomas larger than 4 cm, preferably through computerized tomography (81%), and repeated studies for hormonal hypercortisolism (97%), primary hyperaldosteronism (42%) and pheochromocytoma (76%) over a 4-5 year period (67%). Clinical practice varied among the endocrinologists

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.

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)

Franco Jill

2010-06-01

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.

Radioactive waste disposal in the Gorleben salt deposit

International Nuclear Information System (INIS)

Gizycki, P. von

1985-01-01

In the opinion of five experts, the protective function of the overlying rock as a barrier has turned out to be questionable after borings and measurements carried through at Gorleben. Moreover, the results have also raised doubts about the geological safety of the salt deposit as a barrier in the long run. The geological multibarrier concept must be discarded. Not only critics, but also 3 advocates from the field of official research on radioactive waste disposal state their opinion. (DG) [de

Cerebral salt wasting following traumatic brain injury

Directory of Open Access Journals (Sweden)

Peter Taylor

2017-04-01

Full Text Available Hyponatraemia is the most commonly encountered electrolyte disturbance in neurological high dependency and intensive care units. Cerebral salt wasting (CSW is the most elusive and challenging of the causes of hyponatraemia, and it is vital to distinguish it from the more familiar syndrome of inappropriate antidiuretic hormone (SIADH. Managing CSW requires correction of the intravascular volume depletion and hyponatraemia, as well as mitigation of on-going substantial sodium losses. Herein we describe a challenging case of CSW requiring large doses of hypertonic saline and the subsequent substantial benefit with the addition of fludrocortisone.

Conditioning matrices from high level waste resulting from pyrochemical processing in fluorine salt

International Nuclear Information System (INIS)

Grandjean, Agnes; Advocat, Thierry; Bousquet, Nicolas; Jegou, Christophe

2007-01-01

Separating the actinides from the fission products through reductive extraction by aluminium in a LiF/AlF 3 medium is a process investigated for pyrometallurgical reprocessing of spent fuel. The process involves separation by reductive salt-metal extraction. After dissolving the fuel or the transmutation target in a salt bath, the noble metal fission products are first extracted by contacting them with a slightly reducing metal. After extracting the metal fission products, then the actinides are selectively separated from the remaining fission products. In this hypothesis, all the unrecoverable fission products would be conditioned as fluorides. Therefore, this process will generate first a metallic waste containing the 'reducible' fission products (Pd, Mo, Ru, Rh, Tc, etc.) and a fluorine waste containing alkali-metal, alkaline-earth and rare earth fission products. Immobilization of these wastes in classical borosilicate glasses is not feasible due to the very low solubility of noble metals, and of fluoride in these hosts. Alternative candidates have therefore been developed including silicate glass/ceramic system for fluoride fission products and metallic ones for noble metal fission products. These waste-forms were evaluated for their confinement properties like homogeneity, waste loading, volatility during the elaboration process, chemical durability, etc. using appropriate techniques. (authors)

Study of the incidence and etiology of congenital hypothyroidism in an endemic goiter area after treatment with iodine enriched salt

International Nuclear Information System (INIS)

Liu Shizhen

1992-01-01

A screening program for congenital hypothyroidism (CH) was performed in a severe endemic goiter area, Chengde district including 7 counties, after treatment with Iodine enriched salt, and Beijing city as a control area. From May 1985 to Sep. 1991, 26570 newborns in Beijing city and 16227 in Chengde were screened. The incidence of primary hypothyroidism in Beijing city was 1/8800 and that in Chengde 1/8100. Of all the 5 Ch detected, 3 from Beijing city and 2 from Chengde, were thyroid dysgenesis. Not a single case of endemic goiter cretinism (including both myxedematous and neurological cretinism) was found in our study. We conclude that Iodine deficiency is the only cause of endemic cretinism and this problem can be solved by Iodine enriched salt treatment

UK-Nuclear decommissioning authority and US Salt-stone waste management issues

International Nuclear Information System (INIS)

Lawless, William; Whitton, John

2007-01-01

Available in abstract form only. Full text of publication follows: We update two case studies of stakeholder issues in the UK and US. Earlier versions were reported at Waste Management 2006 and 2007 and at ICEM 2005. UK: The UK nuclear industry has begun to consult stakeholders more widely in recent years. Historically, methods of engagement within the industry have varied, however, recent discussions have generally been carried out with the explicit understanding that engagement with stakeholders will be 'dialogue based' and will 'inform' the final decision made by the decision maker. Engagement is currently being carried out at several levels within the industry; at the national level (via the Nuclear Decommissioning Authority's (NDA) National Stakeholder Group (NSG)); at a local site level (via Site Stakeholder Groups) and at a project level (usually via the Best Practicable Environmental Option process (BPEO)). This paper updates earlier results by the co-author with findings from a second questionnaire issued to the NSG in Phase 2 of the engagement process. An assessment is made regarding the development of stakeholder perceptions since Phase 1 towards the NDA process. US: The US case study reviews the resolution of issues on salt-stone by Department of Energy's (DOE) Savannah River Site (SRS) Citizens Advisory Board (CAB), in Aiken, SC. Recently, SRS-CAB encouraged DOE and South Carolina's regulatory Department of Health and Environmental Control (SC-DHEC) to resolve a conflict preventing SC-DHEC from releasing a draft permit to allow SRS to restart salt-stone operations. It arose with a letter sent from DOE blaming the Governor of South Carolina for delay in restarting salt processing. In reply, the Governor blamed DOE for failing to assure that Salt Waste Processing Facility (SWPF) would be built. SWPF is designed to remove most of the radioactivity from HLW prior to vitrification, the remaining fraction destined for salt-stone. (authors)

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

Directory of Open Access Journals (Sweden)

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.

The advantages of a salt/bentonite backfill for Waste Isolation Pilot Plant disposal rooms

International Nuclear Information System (INIS)

Butcher, B.M.; Novak, C.F.; Jercinovic, M.

1991-04-01

A 70/30 wt% salt/bentonite mixture is shown to be preferable to pure crushed salt as backfill for disposal rooms in the Waste Isolation Pilot Plant (WIPP). This report discusses several selection criteria used to arrive at this conclusion: the need for low permeability and porosity after closure, chemical stability with the surroundings, adequate strength to avoid shear erosion from human intrusion, ease of emplacement, and sorption potential for brine and radionuclides. Both salt and salt/bentonite are expected to consolidate to a final state of impermeability (i.e., ≤ 10 -18 m 2 ) adequate for satisfying federal nuclear regulations. Any advantage of the salt/bentonite mixture is dependent upon bentonite's potential for sorbing brine and radionuclides. Estimates suggest that bentonite's sorption potential for water in brine is much less than for pure water. While no credit is presently taken for brine sorption in salt/bentonite backfill, the possibility that some amount of inflowing brine would be chemically bound is considered likely. Bentonite may also sorb much of the plutonium, americium, and neptunium within the disposal room inventory. Sorption would be effective only if a major portion of the backfill is in contact with radioactive brine. Brine flow from the waste out through highly localized channels in the backfill would negate sorption effectiveness. Although the sorption potentials of bentonite for both brine and radionuclides are not ideal, they are distinctly beneficial. Furthermore, no detrimental aspects of adding bentonite to the salt as a backfill have been identified. These two observations are the major reasons for selecting salt/bentonite as a backfill within the WIPP. 39 refs., 16 figs., 6 tabs

Numerical analysis of impurity separation from waste salt by investigating the change of concentration at the interface during zone refining process

Science.gov (United States)

Choi, Ho-Gil; Shim, Moonsoo; Lee, Jong-Hyeon; Yi, Kyung-Woo

2017-09-01

The waste salt treatment process is required for the reuse of purified salts, and for the disposal of the fission products contained in waste salt during pyroprocessing. As an alternative to existing fission product separation methods, the horizontal zone refining process is used in this study for the purification of waste salt. In order to evaluate the purification ability of the process, three-dimensional simulation is conducted, considering heat transfer, melt flow, and mass transfer. Impurity distributions and decontamination factors are calculated as a function of the heater traverse rate, by applying a subroutine and the equilibrium segregation coefficient derived from the effective segregation coefficients. For multipass cases, 1d solutions and the effective segregation coefficient obtained from three-dimensional simulation are used. In the present study, the topic is not dealing with crystal growth, but the numerical technique used is nearly the same since the zone refining technique was just introduced in the treatment of waste salt from nuclear power industry because of its merit of simplicity and refining ability. So this study can show a new application of single crystal growth techniques to other fields, by taking advantage of the zone refining multipass possibility. The final goal is to achieve the same high degree of decontamination in the waste salt as in zone freezing (or reverse Bridgman) method.

HAW project. Demonstrative disposal of high-level radioactive wastes in the Asse salt mine

International Nuclear Information System (INIS)

Rothfuchs, T.; Duijves, K.; Stippler, R.

1988-01-01

Since 1968 the GSF has been carrying out research and development programs for the final disposal of high-level radioactive waste (HAW) in salt formations. The heat producing waste has been simulated so far by means of electrical heaters and also cobalt-60-sources. In order to improve the final concept for HAW disposal in salt formations the complete technical system of an underground repository is to be tested in an one-to-one scale test facility. To satisfy the test objectives thirty high radioactive canisters containing the radionuclides Cs-137 and Sr-90 will be emplaced in six boreholes located in two test galleries at the 800 m-level in the Asse salt mine. The duration of testing will be approximately five years. For the handling of the radioactive canisters and their emplacement into the boreholes a system consisting of transportation casks, transportation vehicle, disposal machine, and borehole slider will be developed and tested. The actual scientific investigation program is based on the estimation and observation of the interaction between the radioactive canisters and the rock salt. This program includes measurement of thermally and radiolytically induced water and gas release from the rock salt and the radiolytical decomposition of salt minerals. Also the thermally induced stress and deformation fields in the surrounding rock mass will be investigated carefully. The project is funded by the BMFT and the CEC and carrier out in close co-operation with the Netherlands Energy Research Foundation (ECN)

Waste segregation analysis for salt well pumping in the 200 W Area -- Task 3.4

International Nuclear Information System (INIS)

Reynolds, D.A.

1995-01-01

There is an estimated 7 million liters (1.9 million gallons) of potentially complexed waste that need to be pumped from single-shell tanks (SST) in the 200 West Area. This represents up to 40% of the salt well liquor that needs to be pumped in the 200 West Area. There are three double-shell (DST) tanks in the 241-SY tank farm in the 200 West Area. Tank 241-SY-101 is full and not usable. Tank 241-SY-102 has a transuranic (TRU) sludge in the bottom. Current rules prohibit mixing complexed waste with TRU waste. Tank 241-SY-103 has three major problems. First, 241-SY-103 is on the Flammable Watch list. Second, adding waste to tank 241-SY-103 has the potential for an episodic release of hydrogen gas. Third, 241-SY-103 will not hold all of the potentially complexed waste from the SSTs. This document looks at more details regarding the salt well pumping of the 200 West Area tank farm. Some options are considered but it is beyond the scope of this document to provide an in-depth study necessary to provide a defensible solution to the complexed waste problem

Waste Isolation Pilot Plant Salt Decontamination Testing

Energy Technology Data Exchange (ETDEWEB)

Rick Demmer; Stephen Reese

2014-09-01

On February 14, 2014, americium and plutonium contamination was released in the Waste Isolation Pilot Plant (WIPP) salt caverns. At the request of WIPP’s operations contractor, Idaho National Laboratory (INL) personnel developed several methods of decontaminating WIPP salt, using surrogate contaminants and also americium (241Am). The effectiveness of the methods is evaluated qualitatively, and to the extent possible, quantitatively. One of the requirements of this effort was delivering initial results and recommendations within a few weeks. That requirement, in combination with the limited scope of the project, made in-depth analysis impractical in some instances. Of the methods tested (dry brushing, vacuum cleaning, water washing, strippable coatings, and mechanical grinding), the most practical seems to be water washing. Effectiveness is very high, and it is very easy and rapid to deploy. The amount of wastewater produced (2 L/m2) would be substantial and may not be easy to manage, but the method is the clear winner from a usability perspective. Removable surface contamination levels (smear results) from the strippable coating and water washing coupons found no residual removable contamination. Thus, whatever is left is likely adhered to (or trapped within) the salt. The other option that shows promise is the use of a fixative barrier. Bartlett Nuclear, Inc.’s Polymeric Barrier System (PBS) proved the most durable of the coatings tested. The coatings were not tested for contaminant entrapment, only for coating integrity and durability.

Polyethylene encapsulatin of nitrate salt wastes: Waste form stability, process scale-up, and economics

International Nuclear Information System (INIS)

Kalb, P.D.; Heiser, J.H. III; Colombo, P.

1991-07-01

A polyethylene encapsulation system for treatment of low-level radioactive, hazardous, and mixed wastes has been developed at Brookhaven National Laboratory. Polyethylene has several advantages compared with conventional solidification/stabilization materials such as hydraulic cements. Waste can be encapsulated with greater efficiency and with better waste form performance than is possible with hydraulic cement. The properties of polyethylene relevant to its long-term durability in storage and disposal environments are reviewed. Response to specific potential failure mechanisms including biodegradation, radiation, chemical attack, flammability, environmental stress cracking, and photodegradation are examined. These data are supported by results from extensive waste form performance testing including compressive yield strength, water immersion, thermal cycling, leachability of radioactive and hazardous species, irradiation, biodegradation, and flammability. The bench-scale process has been successfully tested for application with a number of specific ''problem'' waste streams. Quality assurance and performance testing of the resulting waste form confirmed scale-up feasibility. Use of this system at Rocky Flats Plant can result in over 70% fewer drums processed and shipped for disposal, compared with optimal cement formulations. Based on the current Rocky Flats production of nitrate salt per year, polyethylene encapsulation can yield an estimated annual savings between $1.5 million and $2.7 million, compared with conventional hydraulic cement systems. 72 refs., 23 figs., 16 tabs

The HAW project. Demonstrative disposal of high-level radioactive wastes in the Asse salt mine

International Nuclear Information System (INIS)

Rothfuchs, T.; Duijves, K.

1988-04-01

Since 1968 the GSF has been carrying out research and development programs for the final disposal of high-level radioactive waste (HAW) in salt formations. The heat producing waste has been simulated so far by means of electrical heaters and also cobalt-60-sources. In order to improve the final concept for HAW disposal in salt formations the complete technical system of an underground repository is to be tested in a one-to-one scale test facility. To satisfy the test objectives thirty high radioactive canisters containing the radionuclides Cs-137 and Sr-90 will be emplaced in six boreholes located in two test galleries at the 800 m-level in the Asse salt mine. The duration of testing will be approximately five years. For the handling of the radioactive canisters and their emplacement into the boreholes a system consisting of transportation casks, transportation vehicle, disposal machine, and borehole slider will be developed and tested. The actual scientific investigation program is based on the estimation and observation of the interaction between the radioactive canisters and the rock salt. This program includes measurement of thermally and radiolytically induced water and gas release from the rock salt and the radiolytical decomposition of salt minerals. Also the thermally induced stress and deformation fields in the surrounding rock mass will be investigated carefully. (orig./HP)

Adrenal Fatigue

Science.gov (United States)

... Search Featured Resource New Mobile App DOWNLOAD Adrenal Fatigue October 2017 Download PDFs English Editors Irina Bancos, MD Additional Resources Mayo Clinic What is adrenal fatigue? The term “adrenal fatigue” has been used to ...

Permeability of natural rock salt from the Waste Isolation Pilot Plant (WIPP) during damage evolution and healing

International Nuclear Information System (INIS)

Pfeifle, T.W.; Hurtado, L.D.

1998-06-01

The US Department of Energy has developed the Waste Isolation Pilot Plant (WIPP) in the bedded salt of southeastern New Mexico to demonstrate the safe disposal of radioactive transuranic wastes. Four vertical shafts provide access to the underground workings located at a depth of about 660 meters. These shafts connect the underground facility to the surface and potentially provide communication between lithologic units, so they will be sealed to limit both the release of hazardous waste from and fluid flow into the repository. The seal design must consider the potential for fluid flow through a disturbed rock zone (DRZ) that develops in the salt near the shafts. The DRZ, which forms initially during excavation and then evolves with time, is expected to have higher permeability than the native salt. The closure of the shaft openings (i.e., through salt creep) will compress the seals, thereby inducing a compressive back-stress on the DRZ. This back-stress is expected to arrest the evolution of the DRZ, and with time will promote healing of damage. This paper presents laboratory data from tertiary creep and hydrostatic compression tests designed to characterize damage evolution and healing in WIPP salt. Healing is quantified in terms of permanent reduction in permeability, and the data are used to estimate healing times based on considerations of first-order kinetics

Disposal of high-level waste from nuclear power plants in Denmark. Salt dome investigations. v.4

International Nuclear Information System (INIS)

1981-01-01

The present report deals with construction, operation and sealing of disposal facilities for high-level waste in a salt dome. It is volume 4 of five volumes that together constitute the final report on the Danish utilities' salt dome investigations. The safety investigations were carried out for a deep-hole disposal facility located in the salt dome on Mors. In principle the results of the investigations also apply to a shaft/mine disposal facility. The facility is designed for the disposal of vitrified high-level waste in the shape of glass canisters. There is a low concentration of waste in each canister, approx. 10%. Furthermore, it was selected to place the waste in an intermediate storage for about 40 years prior to its final disposal. Consequently, heat generation in the waste at the time of final disposal will be modest, resulting in low temperature increase in the salt. As an example, the highest temperature increase will be approx. 40 deg. C. and it will occur at the edge of the hole five years after disposal has taken place. Prior to disposal, the glass canisters are encased in steel casks with 15 cm thick walls. Three canisters are placed in each cask, and 215 casks are stacked on top on one another in each deep-hole from a depth of 1200 m to 2500 m underground. The additional encasing is designed to protect the glass from dissolution should any brine reach the disposal facility. Furthermore, the steel cask protects the glass canisters against pressure from the wall of the hole. The technical design of the disposal facility gives it a considerable safety margin against unexpected events. The investigations proved Cretaceous strata to constitute an effective secondary barrier that would prevent radioactive matter from travelling from the underlying disposal facility to the biosphere. (BP)

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.

Temperature calculations on different configurations for disposal of high-level reprocessing waste in a salt dome model

International Nuclear Information System (INIS)

Hamstra, J.; Kevenaar, J.W.A.M.

1978-06-01

A medium size salt dome is considered as a structure in which a repository can be located for all radioactive wastes to be produced within the scope of a postulated nuclear power program. A dominating design factor for the lay-out of such a waste repository is the temperature distribution in the salt dome resulting from decay heat released from the buried solidified high-level reprocessing waste. Two numerical models are presented for the calculation of both global and local rock salt temperatures. The results of calculations performed with these models are demonstrated to be compatible. Rock salt temperatures related to several types of burial configurations, ranging from two layer configurations with various vertical distances between the layers via a three and a four layer repository to deep bore hole concepts varying from 100 to 600 m bore hole depth, can therefore be calculated with one rather simple unit cell model. The results of these calculations indicate that rock salt temperatures can be kept within acceptable limits to realize a repository using standard mining techniques. The temperatures at mine galery level prove to be a dominating factor in the selection of a repository configuration. More detailed calculations of these temperatures taking into account the loading sequence and the cooling capacity of the mine ventilation are recommended. Finally the apparent advantages of a deep bore hole concept emphasize the need for R and D work with respect to advanced drilling techniques in order to achieve deep dry disposal bore holes that can be realized from a burial mine in the salt dome. (Auth.)

Nuclear waste repository simulation experiments, Asse Salt Mine, Federal Republic of Germany. Annual report, 1983

International Nuclear Information System (INIS)

Rothfuchs, T.; Luebker, D.; Coyle, A.; Kalia, H.

1984-10-01

This is the First Annual report (1983) which describes experiments simulating a nuclear waste respository at the 800-meter level of the Asse Salt Mine in the Federal Republic of Germany. The report describes the test equipment, the Asse Salt Mine, the pretest properties of the salt in the test gallery, and the mine proper. Also included are test data for the first six months of operations on brine migration rates, room closure rates, extensometer readings, stress measurements, and thermal mechanical behavior of the salt. The duration of the experiments will be two years, ending in December 1985. 3 references, 34 figures, 13 tables

The function of packing materials in a high-level nuclear waste repository and some candidate materials: Salt Repository Project

International Nuclear Information System (INIS)

Bunnell, L.R.; Shade, J.W.

1987-03-01

Packing materials should be included in waste package design for a high-level nuclear waste repository in salt. A packing material barrier would increase confidence in the waste package by alleviating possible shortcomings in the present design and prolonging confinement capabilities. Packing materials have been studied for uses in other geologic repositories; appropriately chosen, they would enhance the confinement capabilities of salt repository waste packages in several ways. Benefits of packing materials include retarding or chemically modifying brines to reduce corrosion of the waste package, providing good thermal conductivity between the waste package and host rock, retarding or absorbing radionuclides, and reducing the massiveness of the waste package. These benefits are available at low percentage of total repository cost, if the packing material is properly chosen and used. Several candidate materials are being considered, including oxides, hydroxides, silicates, cement-based mixtures, and clay mixtures. 18 refs

Cerebral salt wasting: a report of three cases

International Nuclear Information System (INIS)

Younas, H.; Sabir, O.; Tarif, N.

2015-01-01

Hyponatremia secondary to the Syndrome of Inappropriate Anti-Diuretic Hormone (SIADH) secretion is commonly observed in patients with various neurological disorders. Cerebral Salt Wasting (CSW) resulting in hyponatremia is also an infrequent occurrence in some patients with neurological disorders. Confusion in differentiating CSW from SIADH may arise since both results in similar electrolyte disturbances. Herein, we report three cases of CSW with intracranial afflictions. CSW was diagnosed on the basis of fractional excretion of urinary sodium and uric acid along with extremely low serum uric acid. Improvements in serum sodium levels after saline hydration and fludrocortisone administration further supported the diagnosis. (author)

Modeling Solute Thermokinetics in LiCI-KCI Molten Salt for Nuclear Waste Separation

Energy Technology Data Exchange (ETDEWEB)

Morgan, Dane; Eapen, Jacob

2013-10-01

Recovery of actinides is an integral part of a closed nuclear fuel cycle. Pyrometallurgical nuclear fuel recycling processes have been developed in the past for recovering actinides from spent metallic and nitride fuels. The process is essentially to dissolve the spent fuel in a molten salt and then extract just the actinides for reuse in a reactor. Extraction is typically done through electrorefining, which involves electrochemical reduction of the dissolved actinides and plating onto a cathode. Knowledge of a number of basic thermokinetic properties of salts and salt-fuel mixtures is necessary for optimizing present and developing new approaches for pyrometallurgical waste processing. The properties of salt-fuel mixtures are presently being studied, but there are so many solutes and varying concentrations that direct experimental investigation is prohibitively time consuming and expensive (particularly for radioactive elements like Pu). Therefore, there is a need to reduce the number of required experiments through modeling of salt and salt-fuel mixture properties. This project will develop first-principles-based molecular modeling and simulation approaches to predict fundamental thermokinetic properties of dissolved actinides and fission products in molten salts. The focus of the proposed work is on property changes with higher concentrations (up to 5 mol%) of dissolved fuel components, where there is still very limited experimental data. The properties predicted with the modeling will be density, which is used to assess the amount of dissolved material in the salt; diffusion coefficients, which can control rates of material transport during separation; and solute activity, which determines total solubility and reduction potentials used during electrorefining. The work will focus on La, Sr, and U, which are chosen to include the important distinct categories of lanthanides, alkali earths, and actinides, respectively. Studies will be performed using LiCl-KCl salt

An experimental study on Sodalite and SAP matrices for immobilization of spent chloride salt waste

Science.gov (United States)

Giacobbo, Francesca; Da Ros, Mirko; Macerata, Elena; Mariani, Mario; Giola, Marco; De Angelis, Giorgio; Capone, Mauro; Fedeli, Carlo

2018-02-01

In the frame of Generation IV reactors a renewed interest in pyro-processing of spent nuclear fuel is underway. Molten chloride salt waste arising from the recovering of uranium and plutonium through pyro-processing is one of the problematic wastes for direct application of vitrification or ceramization. In this work, Sodalite and SAP have been evaluated and compared as potential matrices for confinement of spent chloride salt waste coming from pyro-processing. To this aim Sodalite and SAP were synthesized both in pure form and mixed with different glass matrices, i.e. commercially available glass frit and borosilicate glass. The confining matrices were loaded with mixed chloride salts to study their retention capacities with respect to the elements of interest. The matrices were characterized and leached for contact times up to 150 days at room temperature and at 90 °C. SEM analyses were also performed in order to compare the matrix surface before and after leaching. Leaching results are discussed and compared in terms of normalized releases with similar results reported in literature. According to this comparative study the SAP matrix with glass frit binder resulted in the best matrix among the ones studied, with respect to retention capacities for both matrix and spent fuel elements.

A three-year-old boy with X-linked adrenoleukodystrophy and congenital pulmonary adenomatoid malformation: a case report

Directory of Open Access Journals (Sweden)

Cakan Nedim

2009-12-01

Full Text Available Abstract Introduction X-linked adrenoleukodystrophy leads to demyelination of the nervous system, adrenal insufficiency, and accumulation of long-chain fatty acids. Most young patients with X-linked adrenoleukodystrophy develop seizures and progressive neurologic deficits, and die within the first two decades of life. Congenital or acquired disorders of the respiratory system have not been previously described in patients with X-linked adrenoleukodystrophy. Case presentation A 3-year-old Arabic boy from Yemen presented with discoloration of the mucous membranes and nail beds, which were considered cyanoses due to methemoglobinemia. He also had shortness of breath, fatigue, emesis and dehydration episodes for which he was admitted to our hospital. Chest radiograph and chest computed tomography scans showed congenital pulmonary adenomatoid malformation. A few weeks before the removal of the malformation, he had a significant episode of hypotension and hypoglycemia. This development required further in-hospital evaluation that led to the diagnosis of adrenal insufficiency and the initiation of treatment with corticosteroids. One year later, he developed seizures and loss of consciousness. Magnetic resonance imaging of his head showed diffuse demyelination secondary to X-linked adrenoleukodystrophy. He was treated with anti-seizure and anti-oxidants, and was referred for bone marrow transplant evaluation. Conclusion The presence of adrenal insufficiency, neurologic deficits and seizures are common manifestations of X-linked adrenoleukodystrophy. The association of congenital lung disease with X-linked adrenoleukodystrophy or Addison's disease has not been described previously.

Impact of Salt Waste Processing Facility Streams on the Nitric-Glycolic Flowsheet in the Chemical Processing Cell

Energy Technology Data Exchange (ETDEWEB)

Martino, C. [Savannah River Site (SRS), Aiken, SC (United States). Savannah River National Lab. (SRNL)

2017-08-08

An evaluation of the previous Chemical Processing Cell (CPC) testing was performed to determine whether the planned concurrent operation, or “coupled” operations, of the Defense Waste Processing Facility (DWPF) with the Salt Waste Processing Facility (SWPF) has been adequately covered. Tests with the nitricglycolic acid flowsheet, which were both coupled and uncoupled with salt waste streams, included several tests that required extended boiling times. This report provides the evaluation of previous testing and the testing recommendation requested by Savannah River Remediation. The focus of the evaluation was impact on flammability in CPC vessels (i.e., hydrogen generation rate, SWPF solvent components, antifoam degradation products) and processing impacts (i.e., acid window, melter feed target, rheological properties, antifoam requirements, and chemical composition).

Adrenal incidentaloma

Directory of Open Access Journals (Sweden)

Arnaldi G.

2000-01-01

Full Text Available Incidentally discovered adrenal masses, or adrenal incidentalomas, have become a common clinical problem owing to wide application of radiologic imaging techniques. This definition encompasses a heterogeneous spectrum of pathologic entities, including primary adrenocortical and medullary tumors, benign or malignant lesions, hormonally active or inactive lesions, metastases, and infections. Once an adrenal mass is detected, the clinician needs to address two crucial questions: is the mass malignant, and is it hormonally active? This article provides an overview of the diagnostic clinical approach and management of the adrenal incidentaloma. Mass size is the most reliable variable to distinguish benign and malignant adrenal masses. Adrenalectomy should be recommended for masses greater than 4.0 cm because of the increased risk of malignancy. Adrenal scintigraphy has proved useful in discriminating between benign and malignant lesions. Finally, fine-needle aspiration biopsy is an important tool in the evaluation of oncological patients and it may be useful in establishing the presence of metastatic disease. The majority of adrenal incidentalomas are non-hypersecretory cortical adenomas but an endocrine evaluation can lead to the identification of a significant number of cases with subclinical Cushing's syndrome (5-15%, pheochromocytoma (1.5-13% and aldosteronoma (0-7%. The first step of hormonal screening should include an overnight low dose dexamethasone suppression test, the measure of urinary catecholamines or metanephrines, serum potassium and, in hypertensive patients, upright plasma aldosterone/plasma renin activity ratio. Dehydroepiandrosterone sulfate measurement may show evidence of adrenal androgen excess.

Radioactive waste isolation in salt: Peer review of the Golder Associates draft test plan for in situ testing in an exploratory shaft in salt

International Nuclear Information System (INIS)

Hambley, D.F.; Mraz, D.Z.; Unterberter, R.R.

1987-01-01

This report documents the peer review conducted by Argonne National Laboratory of a document entitled ''Draft Test Plan for In Situ Testing in an Exploratory Shaft in Salt,'' prepared for Battelle Memorial Institute's Office of Nuclear Waste Isolation by Golder Associates, Inc. In general, the peer review panelists found the test plan to be technically sound, although some deficiencies were identified. Recommendations for improving the test plan are presented in this review report. A microfiche copy of the following unpublished report is attached to the inside back cover of this report: ''Draft Test Plan for In Situ Testing in an Exploratory Shaft in Salt,'' prepared by Golder Associates, Inc., for Office of Nuclear Waste Isolation, Battelle Memorial Institute, Columbus, Ohio (March 1985)

Generic aspects of salt repositories

International Nuclear Information System (INIS)

Laughon, R.B.

1979-01-01

The history of geological disposal of radioactive wastes in salt is presented from 1957 when a panel of the National Academy of Sciences-National Research Council recommended burial in bedded salt deposits. Early work began in the Kansas, portion of the Permian Basin where simulated wastes were placed in an abandoned salt mine at Lyons, Kansas, in the late 1960's. This project was terminated when the potential effect of nearby solution mining activities could not be resolved. Evaluation of bedded salts resumed a few years later in the Permian Basin in southeastern New Mexico, and search for suitable sites in the 1970's resulted in the formation of the National Waste Terminal Storage Program in 1976. Evaluation of salt deposits in many regions of the United States has been virtually completed and has shown that deposits having the greatest potential for radioactive waste disposal are those of the largest depositional basins and salt domes of the Gulf Coast region

Dechlorination and Stabilization of Molten Salt Waste by Using xSiO2-yAl2O3- zP2O5 at Melting Temperature

International Nuclear Information System (INIS)

Park, Hwanseo; Kim, Intae; Kim, Hwanyoung; Kim, Joonhyung

2007-01-01

Molten salt waste, which is generated from the pyroprocess to separate uranium and trans-uranium elements from spent nuclear fuel, has been interested to researchers in the radioactive waste management. For its final disposal, direct immobilization into a suitable host matrix or indirect solidification by other chemical routes requires the control of chlorides and its volatility since molten salt wastes mainly consist of volatile metal chlorides. Glass-bonded sodalite (Na 6 M 2 Al 6 Si 6 O 24 Cl 2 , 1-5) suggested by Argonne National Laboratory (ANL), to the present, could be a practical solution to the immobilization of this waste, where waste form can be fabricated at about 915 .deg., lower than the melting temperature of many borosilicate glasses ( -1150 .deg.). A wet dechlorination to oxides or a thermal conversion into borate glass was suggested to remove Cl from salt waste (6-7) and it seemed that the preference of radionuclides for the intended chemical conversions or immobilizations described above could be hardly accomplished or failed, except the phosphate precipitation method suggested by Volkovich and his co-workers (8). Our research group suggested a novel method to treat molten salt waste, named GRSS (Gel-Route Stabilization/Solidification) using Si-P-Al system as a gel-forming system. This showed little vaporization during high temperature process and good leach resistance on Cs and Sr. As another method, this study suggested a method to stabilize molten salt wastes by using xSiO 2 -yAl 2 O 3 - zP 2 O 5 material. GRSS method is considered as a 'reaction system' to completely convert salt waste into stable product while the inorganic material used in this study is a stabilizer for salt wastes. Using this material, this study investigated the reactivity on different metal chlorides, thermal stability, leach-resistance and etc

Assessment of Options for the Treatment of Nitrate Salt Wastes at Los Alamos National Laboratory

International Nuclear Information System (INIS)

Robinson, Bruce Alan; Funk, David John; Stevens, Patrice Ann

2016-01-01

This paper summarizes the methodology used to evaluate options for treatment of the remediated nitrate salt waste containers at Los Alamos National Laboratory. The method selected must enable treatment of the waste drums, which consist of a mixture of complex nitrate salts (oxidizer) improperly mixed with sWheat Scoop®1, an organic kitty litter and absorbent (fuel), in a manner that renders the waste safe, meets the specifications of waste acceptance criteria, and is suitable for transport and final disposal in the Waste Isolation Pilot Plant located in Carlsbad, New Mexico. A Core Remediation Team was responsible for comprehensively reviewing the options, ensuring a robust, defensible treatment recommendation. The evaluation process consisted of two steps. First, a prescreening process was conducted to cull the list on the basis for a decision of feasibility of certain potential options with respect to the criteria. Then, the remaining potential options were evaluated and ranked against each of the criteria in a consistent methodology. Numerical scores were established by consensus of the review team. Finally, recommendations were developed based on current information and understanding of the scientific, technical, and regulatory situation. A discussion of the preferred options and documentation of the process used to reach the recommended treatment options are presented.

Assessment of Options for the Treatment of Nitrate Salt Wastes at Los Alamos National Laboratory

Energy Technology Data Exchange (ETDEWEB)

Robinson, Bruce Alan [Los Alamos National Lab. (LANL), Los Alamos, NM (United States); Funk, David John [Los Alamos National Lab. (LANL), Los Alamos, NM (United States); Stevens, Patrice Ann [Los Alamos National Lab. (LANL), Los Alamos, NM (United States)

2016-03-17

This paper summarizes the methodology used to evaluate options for treatment of the remediated nitrate salt waste containers at Los Alamos National Laboratory. The method selected must enable treatment of the waste drums, which consist of a mixture of complex nitrate salts (oxidizer) improperly mixed with sWheat Scoop®1, an organic kitty litter and absorbent (fuel), in a manner that renders the waste safe, meets the specifications of waste acceptance criteria, and is suitable for transport and final disposal in the Waste Isolation Pilot Plant located in Carlsbad, New Mexico. A Core Remediation Team was responsible for comprehensively reviewing the options, ensuring a robust, defensible treatment recommendation. The evaluation process consisted of two steps. First, a prescreening process was conducted to cull the list on the basis for a decision of feasibility of certain potential options with respect to the criteria. Then, the remaining potential options were evaluated and ranked against each of the criteria in a consistent methodology. Numerical scores were established by consensus of the review team. Finally, recommendations were developed based on current information and understanding of the scientific, technical, and regulatory situation. A discussion of the preferred options and documentation of the process used to reach the recommended treatment options are presented.

Comparison of slagging pyrolysis and molten salt incinerators for treating TRU waste at the INEL

International Nuclear Information System (INIS)

1977-11-01

For the comparison, it is assumed that the waste product is required to meet the acceptance criteria of the Waste Isolation Pilot Plant, i.e., low leachability. Slagging pyrolysis incinerates combustible waste and melts noncombustible waste; the resulting slag forms a glass of low leachability. In the molten salt incinerator, combustion occurs at low temperatures with no accumulation of explosive gases, but the waste must have been previously sorted into combustibles and noncombustibles and then shredded. The economics, safety, and technical features are compared. Advantages, disadvantages, and areas of technical uncertainty of the two systems are listed. Development costs and schedules for the two types of incinerators are discussed

Mineral sources of water and their influence on the safe disposal of radioactive wastes in bedded salt deposits

International Nuclear Information System (INIS)

Fallis, S.M.

1973-12-01

With the increased use of nuclear energy, there will be subsequent increases in high-level radioactive wastes such as Sr 90 , Cs 137 , and Pu 239 . Several agencies have considered the safest possible means to store or dispose of wastes in geologic environments such as underground storage in salt deposits, shale beds, abandoned dry mines, and in clay and shale pits. Salt deposits have received the most favorable attention because they exist in dry environments and because of other desirable properties of halite (its plasticity, gamma-ray shielding, heat dissipation ability, low mining cost, and worldwide abundance). Much work has been done on bedded salt deposits, particularly the Hutchinson Salt Member of the Wellington Formation at Lyons, Kansas. Salt beds heated by the decay of the radioactive wastes may release water by dehydration of hydrous minerals commonly present in evaporite sequences or water present in other forms such as fluid inclusions. More than 80 hydrous minerals are known to occur in evaporite deposits. The occurrences, total water contents (up to 63%) and dehydration temperatures (often less that 150 0 C) of these minerals are given. Since it is desirable to dispose of radioactive wastes in a dry environment, care must be taken that large quantities of water are not released through the heating of hydrous minerals. Seventy-four samples from four cores taken at Lyons, Kansas, were analyzed by x-ray diffraction. The minerals detected were halite, anhydrite, gypsum, polyhalite, dolomite, magnesite, quartz, feldspar, and the clay minerals illite, chlorite, kaolinite, vermiculite, smectite, mixed-layer clay, and corrensite (interstratified chlorite-vermiculite). Of these, gypsum, polyhalite and the clay minerals are all capable of releasing water when heated

Mass transport in bedded salt and salt interbeds

International Nuclear Information System (INIS)

Hwang, Y.; Pigford, T.H.; Chambre, P.L.; Lee, W.W.L.

1989-08-01

Salt is the proposed host rock for geologic repositories of nuclear waste in several nations because it is nearly dry and probably impermeable. Although experiments and experience at potential salt sites indicate that salt may contain brine, the low porosity, creep, and permeability of salt make it still a good choice for geologic isolation. In this paper we summarize several mass-transfer and transport analyses of salt repositories. The mathematical details are given in our technical reports

Noninvasive Prenatal Diagnosis of Congenital Adrenal Hyperplasia Using Cell-Free Fetal DNA in Maternal Plasma

Science.gov (United States)

Tong, Yu K.; Yuen, Tony; Jiang, Peiyong; Pina, Christian; Chan, K. C. Allen; Khattab, Ahmed; Liao, Gary J. W.; Yau, Mabel; Kim, Se-Min; Chiu, Rossa W. K.; Sun, Li; Zaidi, Mone

2014-01-01

Context: Congenital adrenal hyperplasia (CAH) is an autosomal recessive condition that arises from mutations in CYP21A2 gene, which encodes for the steroidogenic enzyme 21-hydroxylase. To prevent genital ambiguity in affected female fetuses, prenatal treatment with dexamethasone must begin on or before gestational week 9. Currently used chorionic villus sampling and amniocentesis provide genetic results at approximately 14 weeks of gestation at the earliest. This means that mothers who want to undergo prenatal dexamethasone treatment will be unnecessarily treating seven of eight fetuses (males and three of four unaffected females), emphasizing the desirability of earlier genetic diagnosis in utero. Objective: The objective of the study was to develop a noninvasive method for early prenatal diagnosis of fetuses at risk for CAH. Patients: Fourteen families, each with a proband affected by phenotypically classical CAH, were recruited. Design: Cell-free fetal DNA was obtained from 3.6 mL of maternal plasma. Using hybridization probes designed to capture a 6-Mb region flanking CYP21A2, targeted massively parallel sequencing (MPS) was performed to analyze genomic DNA samples from parents and proband to determine parental haplotypes. Plasma DNA from pregnant mothers also underwent targeted MPS to deduce fetal inheritance of parental haplotypes. Results: In all 14 families, the fetal CAH status was correctly deduced by targeted MPS of DNA in maternal plasma, as early as 5 weeks 6 days of gestation. Conclusions: MPS on 3.6 mL plasma from pregnant mothers could potentially provide the diagnosis of CAH, noninvasively, before the ninth week of gestation. Only affected female fetuses will thus be treated. Our strategy represents a generic approach for noninvasive prenatal testing for an array of autosomal recessive disorders. PMID:24606108

Long term outcomes in 46, XX adult patients with congenital adrenal hyperplasia reared as males.

Science.gov (United States)

Khattab, A; Yau, M; Qamar, A; Gangishetti, P; Barhen, A; Al-Malki, S; Mistry, H; Anthony, W; Toralles, M B; New, Maria I

2017-01-01

Patients with Congenital Adrenal Hyperplasia (CAH) owing to 21-hydroxylase deficiency and whose karyotype is 46, XX are usually assigned to the female gender. Reported herein are the long term outcomes in three patients with CAH whose karyotype is 46, XX and who were reared as males. A retrospective review of three CAH patients with a 46, XX karyotype who were reared as males was conducted. Gender assignment, clinical and biochemical data, pre and post-genitoplasty genital examinations were reviewed. Gender identity was tested by an extensive questionnaire. Gender role, sexual preference, marital status and sexual satisfaction were evaluated by interview. The three patients were genotyped for the CYP21A2 gene confirming the diagnosis of CAH. Owing to genital virilization, cultural preferences for male gender and the lack of newborn screening programs the three patients reported herein were assigned to the male gender at birth before the diagnosis of CAH was established. In adulthood the patients remained significantly virilized. Thorough psychosexual assessments in adulthood revealed well established male gender identities compatible with their male gender assignments at birth. In all three patients, gender role and behavior were consistent with male gender identity including sexual intercourse with female partners. The three patients reported herein revealed that male gender assignment to CAH patients with a 46, XX karyotype may have a successful outcome providing there is strong parental support and expert endocrine care. No standard guidelines have been published for the gender assignment of CAH patients with a 46, XX karyotype and genital ambiguity. More studies concerning gender assignment in CAH patients with a 46, XX karyotype reared as males are needed. Copyright © 2016 Elsevier Ltd. All rights reserved.

Conceptual design of retrieval systems for emplaced transuranic waste containers in a salt bed depository. Final report

International Nuclear Information System (INIS)

Fogleman, S.F.

1980-04-01

The US Department of Energy and the Nuclear Regulatory Commission have jurisdiction over the nuclear waste management program. Design studies were previously made of proposed repository site configurations for the receiving, processing, and storage of nuclear wastes. However, these studies did not provide operational designs that were suitable for highly reliable TRU retrieval in the deep geologic salt environment for the required 60-year period. The purpose of this report is to develop a conceptual design of a baseline retrieval system for emplaced transuranic waste containers in a salt bed depository. The conceptual design is to serve as a working model for the analysis of the performance available from the current state-of-the-art equipment and systems. Suggested regulations would be based upon the results of the performance analyses

Geochemical processes in marine salt deposits: Their significance and their implications in connection with disposal of radioactive waste within salt domes

Energy Technology Data Exchange (ETDEWEB)

Herrmann, A G [Goettingen Univ. (Germany, F.R.). Geochemisches Inst.

1980-01-01

Attempts to effect permanent disposal of radioactive wastes in marine evaporites should do nothing to disturb, either in the short or the long term, the present relative stability of such bodies of rock. It is necessary to take account of all of the geochemical and physico-chemical reactions known to have been involved in the processes which formed the evaporites before proceeding to an acceptable strategy for disposal of radionucleides. These processes can be represented as three kinds of metamorphism: 1. solution metamorphism, 2. thermal metamorphism, 3. dynamic metamorphism. In all of the evaporite occurrences in Germany such processes have been influential in altering, on occasion significantly, the primary mineralogical composition and have also promoted a considerable degree of transposition of material. Given similar geochemical and physico-chemical premises, these metamorphic processes could become effective now or in the future. It is therefore necessary to discuss the following criteria when examining salt domes as permanent repositories of highly radioactive substances: (1) Temperatures <= 90/sup 0/ +- 10/sup 0/C at the contact between waste containers and rock salt; (2) Temperatures <= 75/sup 0/C within zones of carnallite rocks; (3) Immobilisation of high-level waste in crystalline forms whenever possible; (4) Systems of additional safety barriers around the waste containers or the unreprocessed spent fuel elements. The geochemical and physical effectiveness of the barriers within an evaporite environment must be guaranteed. For example: Ni-Ti-alloys, corundum, ceramic, anhydrite.

Reconsolidated Salt as a Geotechnical Barrier

International Nuclear Information System (INIS)

Hansen, Francis D.; Gadbury, Casey

2015-01-01

Salt as a geologic medium has several attributes favorable to long-term isolation of waste placed in mined openings. Salt formations are largely impermeable and induced fractures heal as stress returns to equilibrium. Permanent isolation also depends upon the ability to construct geotechnical barriers that achieve nearly the same high-performance characteristics attributed to the native salt formation. Salt repository seal concepts often include elements of reconstituted granular salt. As a specific case in point, the Waste Isolation Pilot Plant recently received regulatory approval to change the disposal panel closure design from an engineered barrier constructed of a salt-based concrete to one that employs simple run-of-mine salt and temporary bulkheads for isolation from ventilation. The Waste Isolation Pilot Plant is a radioactive waste disposal repository for defense-related transuranic elements mined from the Permian evaporite salt beds in southeast New Mexico. Its approved shaft seal design incorporates barrier components comprising salt-based concrete, bentonite, and substantial depths of crushed salt compacted to enhance reconsolidation. This paper will focus on crushed salt behavior when applied as drift closures to isolate disposal rooms during operations. Scientific aspects of salt reconsolidation have been studied extensively. The technical basis for geotechnical barrier performance has been strengthened by recent experimental findings and analogue comparisons. The panel closure change was accompanied by recognition that granular salt will return to a physical state similar to the halite surrounding it. Use of run-of-mine salt ensures physical and chemical compatibility with the repository environment and simplifies ongoing disposal operations. Our current knowledge and expected outcome of research can be assimilated with lessons learned to put forward designs and operational concepts for the next generation of salt repositories. Mined salt

Reconsolidated Salt as a Geotechnical Barrier

Energy Technology Data Exchange (ETDEWEB)

Hansen, Francis D. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Gadbury, Casey [USDOE Carlsbad Field Office, NM (United States)

2015-11-01

Salt as a geologic medium has several attributes favorable to long-term isolation of waste placed in mined openings. Salt formations are largely impermeable and induced fractures heal as stress returns to equilibrium. Permanent isolation also depends upon the ability to construct geotechnical barriers that achieve nearly the same high-performance characteristics attributed to the native salt formation. Salt repository seal concepts often include elements of reconstituted granular salt. As a specific case in point, the Waste Isolation Pilot Plant recently received regulatory approval to change the disposal panel closure design from an engineered barrier constructed of a salt-based concrete to one that employs simple run-of-mine salt and temporary bulkheads for isolation from ventilation. The Waste Isolation Pilot Plant is a radioactive waste disposal repository for defense-related transuranic elements mined from the Permian evaporite salt beds in southeast New Mexico. Its approved shaft seal design incorporates barrier components comprising salt-based concrete, bentonite, and substantial depths of crushed salt compacted to enhance reconsolidation. This paper will focus on crushed salt behavior when applied as drift closures to isolate disposal rooms during operations. Scientific aspects of salt reconsolidation have been studied extensively. The technical basis for geotechnical barrier performance has been strengthened by recent experimental findings and analogue comparisons. The panel closure change was accompanied by recognition that granular salt will return to a physical state similar to the halite surrounding it. Use of run-of-mine salt ensures physical and chemical compatibility with the repository environment and simplifies ongoing disposal operations. Our current knowledge and expected outcome of research can be assimilated with lessons learned to put forward designs and operational concepts for the next generation of salt repositories. Mined salt

Waste package reference conceptual designs for a repository in salt

International Nuclear Information System (INIS)

1986-02-01

This report provides the reference conceptual waste package designs for the Office of Nuclear Waste Isolation to baseline these designs, thereby establishing the configuration and interface controls necessary, within the Civilian Radioactive Waste Management Program, formerly the National Waste Terminal Storage Program, to proceed in an orderly manner with preliminary design. Included are designs for the current reference defense high-level waste form from the Savannah River Plant, an optimized commercial high-level waste form, and spent fuel which has been disassembled and compacted into a circular bundle containing either 12 pressurized-water reactor or 30 boiling-water reactor assemblies. For compacted spent fuel, it appears economically attractive to standardize the waste package diameter for all fuel types. The reference waste packages consist of the containerized waste form, a low carbon steel overpack, and, after emplacement, a cover of salt. The overpack is a hollow cylinder with a flat head welded to each end. Its design thickness is the sum of the structural thickness required to resist the 15.4-MPa lithostatic pressure plus the corrosion allowance necessary to assure the required structural thickness will exist through the 1000-year containment period. Based on available data and completed analyses, the reference concepts described in this report satisfy all requirements of the US Department of Energy and the US Nuclear Regulatory Commission with reasonable assurance. In addition, sufficient design maturity exists to form a basis for preliminary design; these concepts can be brought under configuration control to serve as reference package designs. Development programs are identified that will be required to support these designs during the licensing process. 19 refs., 37 figs., 31 tabs

A case of adrenal Cushing’s syndrome with bilateral adrenal masses

Directory of Open Access Journals (Sweden)

Ya-Wun Guo

2016-05-01

Full Text Available A functional lesion in corticotrophin (ACTH-independent Cushing’s syndrome is difficult to distinguish from lesions of bilateral adrenal masses. Methods for distinguishing these lesions include adrenal venous sampling and 131I-6β-iodomethyl-19-norcholesterol (131I-NP-59 scintigraphy. We present a case of a 29-year-old Han Chinese female patient with a history of hypercholesterolaemia and polycystic ovary syndrome. She presented with a 6month history of an 8kg body weight gain and gradual rounding of the face. Serial examinations revealed loss of circadian rhythm of cortisol, elevated urinary free-cortisol level and undetectable ACTH level (<5pg/mL. No suppression was observed in both the low- and high-dose dexamethasone suppression tests. Adrenal computed tomography revealed bilateral adrenal masses. Adrenal venous sampling was performed, and the right-to-left lateralisation ratio was 14.29. The finding from adrenal scintigraphy with NP-59 was consistent with right adrenal adenoma. The patient underwent laparoscopic right adrenalectomy, and the pathology report showed adrenocortical adenoma. Her postoperative cortisol level was 3.2μg/dL, and her Cushingoid appearance improved. In sum, both adrenal venous sampling and 131I-NP-59 scintigraphy are good diagnostic methods for Cushing’s syndrome presenting with bilateral adrenal masses.

Identifying suitable piercement salt domes for nuclear waste storage sites

International Nuclear Information System (INIS)

Kehle, R.; e.

1980-08-01

Piercement salt domes of the northern interior salt basins of the Gulf of Mexico are being considered as permanent storage sites for both nuclear and chemically toxic wastes. The suitable domes are stable and inactive, having reached their final evolutionary configuration at least 30 million years ago. They are buried to depths far below the level to which erosion will penetrate during the prescribed storage period and are not subject to possible future reactivation. The salt cores of these domes are themselves impermeable, permitting neither the entry nor exit of ground water or other unwanted materials. In part, a stable dome may be recognized by its present geometric configuration, but conclusive proof depends on establishing its evolutionary state. The evolutionary state of a dome is obtained by reconstructing the growth history of the dome as revealed by the configuration of sedimentary strata in a large area (commonly 3,000 square miles or more) surrounding the dome. A high quality, multifold CDP reflection seismic profile across a candidate dome will provide much of the necessary information when integrated with available subsurface control. Additional seismic profiles may be required to confirm an apparent configuration of the surrounding strata and an interpreted evolutionary history. High frequency seismic data collected in the near vicinity of a dome are also needed as a supplement to the CDP data to permit accurate depiction of the configuration of shallow strata. Such data must be tied to shallow drill hole control to confirm the geologic age at which dome growth ceased. If it is determined that a dome reached a terminal configuration many millions of years ago, such a dome is incapable of reactivation and thus constitutes a stable storage site for nuclear wastes

Nuclear waste repository simulation experiments, Asse salt mine, Federal Republic of Germany. Annual report 1984

International Nuclear Information System (INIS)

Rothfuchs, T.; Feddersen, H.K.; Schwarzianeck, P.; Staupendahl, G.; Coyle, A.J.; Eckert, J.; Kalia, H.

1986-07-01

This is the second joint annual report (1984) on experiments simulating a nuclear waste repository at the 800-m (2624-ft) level of the Asse salt mine in the Federal Republic of Germany. This report describes the Asse salt mine, the test equipment, and the pretest properties of the salt in the mine and in the vicinity of the test area. Also included are test data for the first 19 months of operation on the following: brine migration rates, thermal mechanical behavior of the salt (including room closure, stress reading, and thermal profiles), and borehole gas pressures. In addition to field data, laboratory analyses of results are included in this report. The duration of the experiment will be 2 years, ending in December 1985

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 +

From cerebral salt wasting to diabetes insipidus with adipsia: case report of a child with craniopharyngioma.

Science.gov (United States)

Raghunathan, Veena; Dhaliwal, Maninder Singh; Gupta, Aditya; Jevalikar, Ganesh

2015-03-01

Craniopharyngioma is associated with a wide and interesting variety of sodium states both by itself and following surgical resection. These are often challenging to diagnose, especially given their dynamic nature during the perioperative course. We present the case of a boy with craniopharyngioma who had hyponatremia due to cerebral salt wasting preoperatively, developed diabetes insipidus (DI) intraoperatively and proceeded to develop hypernatremia with adipsic DI. Cerebral salt wasting is a rare presenting feature of craniopharyngioma. Postoperative DI can be associated with thirst abnormalities including adipsia due to hypothalamic damage; careful monitoring and a high index of suspicion are required for its detection. Adipsic DI is a difficult condition to manage; hence a conservative surgical approach is suggested.

Bibliography of studies for the Salt Repository Project Office of the Civilian Radioactive Waste Management Program, April 1978-May 1986

International Nuclear Information System (INIS)

1986-10-01

DOE/CH/10140-05 is an annotated bibliography of approved reports that have been produced for the US Department of Energy Salt Repository Project Office of the Civilian Radioactive Waste Management Program since April 1978. This document is intended for use by the US Department of Energy, State and local officials, the US Nuclear Regulatory Commission, contractors to the Office of Nuclear Waste Isolation, concerned citizens, and others who need a comprehensive listing of reports related to a nuclear waste repository in salt. This document consists of a main report listing, appendixes with Work Breakdown Structure lists, and a topical index

Bibliography of studies for the Salt Repository Project Office of the Civilian Radioactive Waste Management Program, April 1978-December 1986

International Nuclear Information System (INIS)

1987-06-01

This document is an annotated bibliography of approved reports that have been produced for the US Department of Energy Salt Repository Project Office of the Civilian Radioactive Waste Management Program since April 1978. This document is intended for use by the US Department of Energy, State and local officials, the US Nuclear Regulatory Commission, contractors to the Office of Nuclear Waste Isolation, concerned citizens, and others who need a comprehensive listing of reports related to a nuclear waste repository in salt. This document consists of a main report listing, appendixes with Work Breakdown Structure lists, and a topical index

Comparison of the salt domes Asse and Gorleben with regard to their suitability for the final storage of radoactive wastes

International Nuclear Information System (INIS)

Deisenroth, Norbert; Kokorsch, Rudolf

2012-01-01

In Germany, the search for a proper solution to the issue of final disposal of radioactive wastes is complicated by political leaders. The Gorleben moratorium from October 2000 delayed the proper solution unnecessary to ten years. Asse proves that salt domes such as Gorleben do not offer a permanent partitioning of the waste over the biosphere. With this in mind, the authors of the contribution under consideration compare the two salt domes Gorleben and Asse from a mining and geological point of view based on publicly available data with regard to their suitability for the disposal of radioactive waste.

Worth its salt?

Science.gov (United States)

The idea that all underground salt deposits can serve as storage sites for toxic and nuclear waste does not always hold water—literally. According to Daniel Ronen and Brian Berkowitz of Israel's Weizmann Institute of Science and Yoseph Yechieli of the Geological Survey of Israel, some buried salt layers are in fact highly conductive of liquids, suggesting that wastes buried in their confines could easily leech into groundwater and nearby soil.When drilling three wells into a 10,000-year-old salt layer near the Dead Sea, the researchers found that groundwater had seeped into the layer and had absorbed some of its salt.

Prostaglandin-E2 Mediated Increase in Calcium and Phosphate Excretion in a Mouse Model of Distal Nephron Salt Wasting.

Directory of Open Access Journals (Sweden)

Manoocher Soleimani

Full Text Available Contribution of salt wasting and volume depletion to the pathogenesis of hypercalciuria and hyperphosphaturia is poorly understood. Pendrin/NCC double KO (pendrin/NCC-dKO mice display severe salt wasting under basal conditions and develop profound volume depletion, prerenal renal failure, and metabolic alkalosis and are growth retarded. Microscopic examination of the kidneys of pendrin/NCC-dKO mice revealed the presence of calcium phosphate deposits in the medullary collecting ducts, along with increased urinary calcium and phosphate excretion. Confirmatory studies revealed decreases in the expression levels of sodium phosphate transporter-2 isoforms a and c, increases in the expression of cytochrome p450 family 4a isotypes 12 a and b, as well as prostaglandin E synthase 1, and cyclooxygenases 1 and 2. Pendrin/NCC-dKO animals also had a significant increase in urinary prostaglandin E2 (PGE-2 and renal content of 20-hydroxyeicosatetraenoic acid (20-HETE levels. Pendrin/NCC-dKO animals exhibit reduced expression levels of the sodium/potassium/2chloride co-transporter 2 (NKCC2 in their medullary thick ascending limb. Further assessment of the renal expression of NKCC2 isoforms by quantitative real time PCR (qRT-PCR reveled that compared to WT mice, the expression of NKCC2 isotype F was significantly reduced in pendrin/NCC-dKO mice. Provision of a high salt diet to rectify volume depletion or inhibition of PGE-2 synthesis by indomethacin, but not inhibition of 20-HETE generation by HET0016, significantly improved hypercalciuria and salt wasting in pendrin/NCC dKO mice. Both high salt diet and indomethacin treatment also corrected the alterations in NKCC2 isotype expression in pendrin/NCC-dKO mice. We propose that severe salt wasting and volume depletion, irrespective of the primary originating nephron segment, can secondarily impair the reabsorption of salt and calcium in the thick ascending limb of Henle and/or proximal tubule, and reabsorption of

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.

Prevalence of and markers for the attenuated form of congenital adrenal hyperplasia and hyperprolactinemia masquerading as polycystic ovarian disease.

Science.gov (United States)

Benjamin, F; Deutsch, S; Saperstein, H; Seltzer, V L

1986-08-01

To determine the prevalence of the attenuated form of congenital adrenal hyperplasia (CAH) and hyperprolactinemia (HPPN) relative to polycystic ovarian disease (PCOD), 100 consecutive women presenting with the classic clinical features of PCOD were evaluated by basal hormonal profiles and subsequent adrenocorticotropic hormone (ACTH) stimulation tests. The study also sought biochemical markers for CAH other than ACTH stimulation. The prevalences were found to be as follows: PCOD, 65%; PCOD with HPPN, 9%; HPPN, 3%, end-organ hypersensitivity (EOH), 4%; homozygotic CAH, 4%; and heterozygotic CAH, 15%. Other than the differential response to ACTH, the only other biochemical markers observed for homozygotic CAH were significantly higher basal levels of testosterone (T) and 17 alpha-hydroxyprogesterone (17-OHP). Luteinizing hormone/follicle-stimulating hormone ratio, androstenedione, and dehydroepiandrosterone sulfate all showed no significant differences between homozygotic CAH, heterozygotic CAH, HPPN, PCOD, and EOH. This study establishes the relative prevalences of the syndromes commonly mimicking PCOD. We also conclude that the observed low incidence of CAH does not justify routine ACTH testing on all patients presenting with features of PCOD--however, our data suggest that patients with basal serum levels of T and 17-OHP greater than 50% above the upper limit of normal should undergo this dynamic test, especially if there are also certain clinical features suggestive of CAH.

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.

Preliminary area selection considerations for radioactive waste repositories in bedded salt

International Nuclear Information System (INIS)

Wagoner, J.L.; Steinborn, T.L.

1979-01-01

This guide describes an approach to selection of areas of bedded salt which contain potentially suitable sites for the storage of radioactive waste. To evaluate a site selected by a license applicant, it is necessary to understand the technical site characteristics which should be considered in the preliminary phase of site selection. These site characteristics are presented here in checklist form, and each item is accompanied by a discussion which explains its significance. These qualitative considerations are used first to select an area of interest within a broad geologic or geomorphic region. Once an area has been selected, more quantitative information must be acquired to determine whether the proposed site meets the resultations for storage of nuclear waste

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.

Mineral sources of water and their influence on the safe disposal of radioactive wastes in bedded salt deposits

Energy Technology Data Exchange (ETDEWEB)

Fallis, S.M.

1973-12-01

With the increased use of nuclear energy, there will be subsequent increases in high-level radioactive wastes such as Sr/sup 90/, Cs/sup 137/, and Pu/sup 239/. Several agencies have considered the safest possible means to store or dispose of wastes in geologic environments such as underground storage in salt deposits, shale beds, abandoned dry mines, and in clay and shale pits. Salt deposits have received the most favorable attention because they exist in dry environments and because of other desirable properties of halite (its plasticity, gamma-ray shielding, heat dissipation ability, low mining cost, and worldwide abundance). Much work has been done on bedded salt deposits, particularly the Hutchinson Salt Member of the Wellington Formation at Lyons, Kansas. Salt beds heated by the decay of the radioactive wastes may release water by dehydration of hydrous minerals commonly present in evaporite sequences or water present in other forms such as fluid inclusions. More than 80 hydrous minerals are known to occur in evaporite deposits. The occurrences, total water contents (up to 63%) and dehydration temperatures (often less that 150/sup 0/C) of these minerals are given. Since it is desirable to dispose of radioactive wastes in a dry environment, care must be taken that large quantities of water are not released through the heating of hydrous minerals. Seventy-four samples from four cores taken at Lyons, Kansas, were analyzed by x-ray diffraction. The minerals detected were halite, anhydrite, gypsum, polyhalite, dolomite, magnesite, quartz, feldspar, and the clay minerals illite, chlorite, kaolinite, vermiculite, smectite, mixed-layer clay, and corrensite (interstratified chlorite-vermiculite). Of these, gypsum, polyhalite and the clay minerals are all capable of releasing water when heated.

Radioactive waste isolation in salt: special advisory report on the status of the Office of Nuclear Waste Isolation's plans for repository performance assessment

International Nuclear Information System (INIS)

Ditmars, J.D.; Walbridge, E.W.; Rote, D.M.; Harrison, W.; Herzenberg, C.L.

1983-10-01

Repository performance assessment is analysis that identifies events and processes that might affect a repository system for isolation of radioactive waste, examines their effects on barriers to waste migration, and estimates the probabilities of their occurrence and their consequences. In 1983 Battelle Memorial Institute's Office of Nuclear Waste Isolation (ONWI) prepared two plans - one for performance assessment for a waste repository in salt and one for verification and validation of performance assessment technology. At the request of the US Department of Energy's Salt Repository Project Office (SRPO), Argonne National Laboratory reviewed those plans and prepared this report to advise SRPO of specific areas where ONWI's plans for performance assessment might be improved. This report presents a framework for repository performance assessment that clearly identifies the relationships among the disposal problems, the processes underlying the problems, the tools for assessment (computer codes), and the data. In particular, the relationships among important processes and 26 model codes available to ONWI are indicated. A common suggestion for computer code verification and validation is the need for specific and unambiguous documentation of the results of performance assessment activities. A major portion of this report consists of status summaries of 27 model codes indicated as potentially useful by ONWI. The code summaries focus on three main areas: (1) the code's purpose, capabilities, and limitations; (2) status of the elements of documentation and review essential for code verification and validation; and (3) proposed application of the code for performance assessment of salt repository systems. 15 references, 6 figures, 4 tables

A prenatally detected adrenal cyst treated by adrenal-sparing surgery

African Journals Online (AJOL)

A neonatal case of left adrenal cyst detected in utero and successfully treated by adrenal-sparing surgery is presented and discussed with review of the literature. Incidentally discovered prenatal adrenal masses present a diagnostic dilemma. Benign and malignant conditions can present as a fetal suprarenal mass. There is ...

Corrosion of carbon steel in saturated high-level waste salt solutions

International Nuclear Information System (INIS)

Wiersma, B.J.; Parish, W.R.

1997-01-01

High level waste stored as crystallized salts is to be removed from carbon steel tanks by water dissolution. Dissolution of the saltcake must be performed in a manner which will not impact the integrity of the tank. Corrosion testing was performed to determine the amount of corrosion inhibitor that must be added to the dissolution water in order to ensure that the salt solution formed would not induce corrosion degradation of the tank materials. The corrosion testing performed included controlled potential slow strain rate, coupon immersion, and potentiodynamic polarization tests. These tests were utilized to investigate the susceptibility of the cooling coil material to stress corrosion cracking in the anticipated environments. No evidence of SCC was observed in any of the tests. Based on these results, the recommended corrosion requirements were that the temperature of the salt solution be less than 50 degrees C and that the minimum hydroxide concentration be 0.4 molar. It was also recommended that the hydroxide concentration not stay below 0.4 molar for longer than 45 days

Radiant energy dissipation during final storage of high-level radioactive waste in rock salt

International Nuclear Information System (INIS)

Ramthun, H.

1981-08-01

A final disposal concept is assumed where the high-active waste from 1400 t of uranium, remaining after conditioning, is solidified in borosilicate glass and distributed in 1.760 waste casks. These containers 1.2 m in height and 0.3 m in diameter are to be buried 10 years after the fuel is removed from the reactor in the 300 m deep boreholes of a salt dome. For this design the mean absorbed dose rates are calculated in the glass die (3.9 Gy/s), the steel mantle (0.26 Gy/s) and in the salt rock (0.12 Gy/s at a distance of 1 cm and 0.034 Gy/s at a distance of 9 cm from the container surface) valid at the beginning of disposal. The risk involved with these amounts of stored lattice energy is shortly discussed. (orig.) [de

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.

Adrenal neoplasms

International Nuclear Information System (INIS)

Low, G.; Dhliwayo, H.; Lomas, D.J.

2012-01-01

Adenoma, myelolipoma, phaeochromocytoma, metastases, adrenocortical carcinoma, neuroblastoma, and lymphoma account for the majority of adrenal neoplasms that are encountered in clinical practice. A variety of imaging methods are available for evaluating adrenal lesions including ultrasound, computed tomography (CT), magnetic resonance imaging (MRI), and nuclear medicine techniques such as meta-iodobenzylguanidine (MIBG) scintigraphy and positron-emission tomography (PET). Lipid-sensitive imaging techniques such as unenhanced CT and chemical shift MRI enable detection and characterization of lipid-rich adenomas based on an unenhanced CT attenuation of ≤10 HU and signal loss on opposed-phase compared to in-phase T1-weighted images, respectively. In indeterminate cases, an adrenal CT washout study may differentiate adenomas (both lipid-rich and lipid-poor) from other adrenal neoplasms based on an absolute percentage washout of >60% and/or a relative percentage washout of >40%. This is based on the principle that adenomas show rapid contrast washout while most other adrenal neoplasms including malignant tumours show slow contrast washout instead. 18 F-2-fluoro-2-deoxy-D-glucose–PET ( 18 FDG-PET) imaging may differentiate benign from malignant adrenal neoplasms by demonstrating high tracer uptake in malignant neoplasms based on the increased glucose utilization and metabolic activity found in most of these malignancies. In this review, the multi-modality imaging appearances of adrenal neoplasms are discussed and illustrated. Key imaging findings that facilitate lesion characterization and differentiation are emphasized. Awareness of these imaging findings is essential for improving diagnostic confidence and for reducing misinterpretation errors.

Phase Equilibrium Studies of Savannah River Tanks and Feed Streams for the Salt Waste Processing Facility

Energy Technology Data Exchange (ETDEWEB)

Weber, C.F.

2001-06-19

A chemical equilibrium model is developed and used to evaluate supersaturation of tanks and proposed feed streams to the Salt Waste Processing Facility. The model uses Pitzer's model for activity coefficients and is validated by comparison with a variety of thermodynamic data. The model assesses the supersaturation of 13 tanks at the Savannah River Site (SRS), indicating that small amounts of gibbsite and or aluminosilicate may form. The model is also used to evaluate proposed feed streams to the Salt Waste Processing Facility for 13 years of operation. Results indicate that dilutions using 3-4 M NaOH (about 0.3-0.4 L caustic per kg feed solution) should avoid precipitation and reduce the Na{sup +} ion concentration to 5.6 M.

A reactive distillation process for the treatment of LiCl-KCl eutectic waste salt containing rare earth chlorides

Energy Technology Data Exchange (ETDEWEB)

Eun, H.C., E-mail: ehc2004@kaeri.re.kr; Choi, J.H.; Kim, N.Y.; Lee, T.K.; Han, S.Y.; Lee, K.R.; Park, H.S.; Ahn, D.H.

2016-11-15

The pyrochemical process, which recovers useful resources (U/TRU metals) from used nuclear fuel using an electrochemical method, generates LiCl-KCl eutectic waste salt containing radioactive rare earth chlorides (RECl{sub 3}). It is necessary to develop a simple process for the treatment of LiCl-KCl eutectic waste salt in a hot-cell facility. For this reason, a reactive distillation process using a chemical agent was achieved as a method to separate rare earths from the LiCl-KCl waste salt. Before conducting the reactive distillation, thermodynamic equilibrium behaviors of the reactions between rare earth (Nd, La, Ce, Pr) chlorides and the chemical agent (K{sub 2}CO{sub 3}) were predicted using software. The addition of the chemical agent was determined to separate the rare earth chlorides into an oxide form using these equilibrium results. In the reactive distillation test, the rare earth chlorides in LiCl-KCl eutectic salt were decontaminated at a decontamination factor (DF) of more than 5000, and were mainly converted into oxide (Nd{sub 2}O{sub 3}, CeO{sub 2}, La{sub 2}O{sub 3}, Pr{sub 2}O{sub 3}) or oxychloride (LaOCl, PrOCl) forms. The LiCl-KCl was purified into a form with a very low concentration (<1 ppm) for the rare earth chlorides.

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 ...

Evaluation of iron-base materials for waste package containers in a salt repository

International Nuclear Information System (INIS)

Westerman, R.E.; Nelson, J.L.; Kuhn, W.L.; Basham, S.G.; Moak, D.A.; Pitman, S.G.

1983-11-01

Design studies for high-level nuclear waste packages for salt repositories have identified low-carbon steel as a candidate material for containers. Among the requirements are strength, corrosion resistance, and fabricability. The studies of the corrosion resistance and structural stability of iron-base materials (particularly low-carbon steel) are treated in this paper. The materials have been exposed in brines that are characteristic of the potential sites for salt repositories. The effects of temperature, radiation level, oxygen level and other parameters are under investigation. The initial development of corrosion models for these environments is presented with discussion of the key mechanisms under consideration. 6 references, 5 figures

[Addison's disease : Primary adrenal insufficiency].

Science.gov (United States)

Pulzer, A; Burger-Stritt, S; Hahner, S

2016-05-01

Adrenal insufficiency, a rare disorder which is characterized by the inadequate production or absence of adrenal hormones, may be classified as primary adrenal insufficiency in case of direct affection of the adrenal glands or secondary adrenal insufficiency, which is mostly due to pituitary or hypothalamic disease. Primary adrenal insufficiency affects 11 of 100,000 individuals. Clinical symptoms are mainly nonspecific and include fatigue, weight loss, and hypotension. The diagnostic test of choice is dynamic testing with synthetic ACTH. Patients suffering from chronic adrenal insufficiency require lifelong hormone supplementation. Education in dose adaption during physical and mental stress or emergency situations is essential to prevent life-threatening adrenal crises. Patients with adrenal insufficiency should carry an emergency card and emergency kit with them.

Heat transfer analysis of the waste-container sleeve/salt configuration

International Nuclear Information System (INIS)

Callahan, G.D.; Ratigan, J.L.; Russell, J.E.; Fossum, A.F.

1975-01-01

Prior to this investigation, the heat transport considered was only that of straight conduction. The waste container, air gap, and sleeve arrangement was considered to be a single, consistent, time-dependent, heat-generating unit in intimate contact with the salt. The conduction model does not accurately model the heat transfer mechanisms available. Thus radiation and combined radiation and convection must also be considered in the determination of the temperature field. As would be expected, the canister temperatures are higher for the case of radiation across the airgap than those that result from conduction when the canister is in intimate contact with the salt. For the radiation case, the canister temperatures rise rapidly to a temperature of approximately 1,140 0 F and maintain an almost steady state condition for one year whereafter the temperatures slowly decrease. The far field temperatures, near the pillar centerline, are essentially equivalent for all cases. As time proceeds, the far field temperatures of the conduction models are about 15% different

The dispersal and impact of salt from surface storage piles the Waste Isolation Pilot Plant

International Nuclear Information System (INIS)

Reith, C.C.; Louderbough, E.T.

1986-01-01

A comprehensive program of ecological studies occurs at the Waste Isolation Pilot Plant (WIPP) in an effort to detect and quantify impacts of excavated salt which is stored on the surface in two piles: one having originated in 1980, the other in 1984. Both piles are surrounded by berms which channel runoff to holding ponds, so nearly all dispersal is due to the resuspension, transport, and deposition of salt particles by wind. Ecological parameters which have been monitored since 1984 include: visual evidence (via photography), soil properties, microbial activity, leaf-litter decomposition, seedling emergence, plant foliar cover, and plant species diversity. These are periodically assessed at experimental plots near the salt piles, and at control plots several kilometers away

Areal thermal loading recommendations for nuclear waste repositories in salt

International Nuclear Information System (INIS)

Russell, J.E.

1979-06-01

This document gives a wider understanding of the history of the recommended thermal loadings in salt for both high-level waste (HLW) from fresh UO 2 -fueled, light-water reactors (LWR) with no recycle and spent unreprocessed fuel (SURF) from LWRs. Aspects of the current recommendations that need further study are identified. Finally, an interim set of design thermal-loading recommendations are given that have a common rationale of satisfying performance limits within our current state of knowledge. These recommendations are made on a generic rather than a site-specific basis. 11 figures, 5 tables

ICP-MS nebulizer performance for analysis of SRS high salt simulated radioactive waste tank solutions (number-sign 3053)

International Nuclear Information System (INIS)

Jones, V.D.

1997-01-01

High Level Radioactive Waste Tanks at the Savannah River Site are high in salt content. The cross-flow nebulizer provided the most stable signal for all salt matrices with the smallest signal loss/suppression due to this matrix. The DIN exhibited a serious lack of tolerance for TDS; possibly due to physical de-tuning of the nebulizer efficiency

Calculations on the development in space and time of the temperature field around a repository of medium and high active wastes in a salt formation

International Nuclear Information System (INIS)

Delisle, G.

1980-01-01

The concept of nuclear waste disposal of th of the Federal Republic of Germany calls for the burial of the wastes within a salt formation. A small portion of the wastes will generate heat after the disposal procedure. A temperature rise within the salt formation, in space and time limited, will be the consequence. The temperature change at any point in the near or far field of the disporal area can be calculated with the aid of numerical models. The thermal parameters representative for the bulk material of the Zechstein formation in NW-Germany, on which the calculations are based, will be discussed in detail. The interrelation between the concentration of heat producing wastes in the disposal field and the maximum average temperature in the salt formation will be treated. By defining numerical models, which are based on assumed shapes of a salt dome and a disposal area, the temperature development in the near and far field of a nuclear repository are shown. (orig.) [de

Sirenomelia associated with discoid adrenal and lumbar meningocoele: An autopsy report.

Science.gov (United States)

Islam, Nelofar; Mandal, Bappa; Das, Ram Narayan; Bera, Goutam; Mukherjee, Suchandra; Chatterjee, Uttara

2017-11-01

Mermaid syndrome or Sirenomelia is a rare congenital deformity in which the legs are fused and bears resemblance to mermaid's tail. It carries a poor prognosis, due to associated urogenital and gastrointestinal abnormalities. An early antenatal diagnosis using Magnetic Resonance Imaging (MRI) can help in termination of pregnancy. Embryologically, it is considered as the extreme form of caudal regression syndrome due to the persistence of vitelline artery. Here, we report a case of Sirenomelia associated with bilateral renal agenesis along with the rare findings of discoid adrenal, lumbar meningocoele and abnormalities of the hand. Copyright © 2017 Elsevier GmbH. All rights reserved.

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...

Vitrification in the presence of salts