salt-wasting congenital adrenal: Topics by WorldWideScience.org

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

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...Full Text Available

2010-01-01

Should Male Gender Assignment be Considered in the Markedly Virilized Patient With 46,XX and Congenital Adrenal Hyperplasia?

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...Full Text Available

2010-01-01

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Purpose 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. Materials and Methods 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. Results 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 ...

2010-01-01

PAX6 Mutations and Clinical Features of Congenital Aniridia

Clinical features of korean patients with congenital aniridia.

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2008-11-01

A congenital lethal form of hypophosphatasia: histologic and ultrastructural study.

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2010-10-01

Retroperitoneoscopic partial adrenalectomy for small adrenal tumours (<=1 cm): the Ruijin clinical experience in 88 patients

PMID:8677780

1996-05-01

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Study Type - Therapy (case series)Level of Evidence 4 OBJECTIVE To present our experience of retroperitoneoscopic partial adrenalectomy (RPA) for small adrenal tumours, as with modern imaging methods small adrenal lesions are being diagnosed more commonly, and retroperitoneoscopic adrenal surgery for small adrenal tumours (PATIENTS AND METHODS We retrospectively reviewed the records of 389 consecutive retroperitoneoscopic adrenalectomies from September 2005 to December 2008, 88 of which were small adrenal tumours and treated by RPA. Ultrasonography and computed tomography (CT) were used in all patients before RPA, and magnetic resonance imaging or positron emission tomography/CT in some patients. We used RPA for adrenal tumours and total adrenalectomy for adrenal cancer. During the surgery...

2010-01-01

Nonchylous idiopathic pleural effusion in the newborn

Congenital Lobar Emphysema

Congenital isolated pleural effusion is a rare cause of respiratory distress in neonates. It is usually chylous. Herein, we report a rare case of nonchylous congenital idiopathic pleural effusion.

2011-01-01

Potassium transport in adrenal mitochondria; Charakterystyka transportu potasu w mitochondriach nadnerczy

SummaryCongenital lobar emphysema (CLE) characterized by over distension and air-trapping in the affected lobe is one of the causes of infantile respiratory distress requiring...Full Text Available

2009-08-01

Naltrexone Inhibits Catecholamine Secretion Evoked by Nicotinic Receptor Stimulation in the Perfused Rat Adrenal Medulla

Short communication.

1995-12-31

A prospective study on the incidence and predictive factors of relative adrenal insufficiency in Korean critically-ill patients.

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2005-08-01

Importance of the Adrenal Gland Blood Supply During Laparoscopic Subtotal Adrenalectomy

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2009-08-01

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Abstract Introduction: Laparoscopic subtotal adrenalectomy (LSA) has emerged as a treatment option in the management of certain adrenal pathologies. After LSA, a satisfactory blood supply to the adrenal remnant is vital to preserve steroid function. The aim of this study was to review the adrenal blood supply with relevance to LSA. Materials and Methods: The adrenal blood supply was examined in three ways: 1) a review of six previous cadaver studies looking at adrenal blood supply; 2) a review of our 164 consecutive laparoscopic adrenalectomies (LAs) (January 1999?January 2009); the details of the main adrenal veins and variants had been prospectively recorded; and 3) dissection of 4 cadaver specimens (macroscopic and histologic examination was performed). Results: The six cadaver studies ...

2010-01-01

Computerized tomography of adrenal glands in the investigation of Cushing's syn

Role of congenital rubella reference laboratory: 21-months-surveillance in Liguria, Italy.

Computerized tomography of the adrenal glands was performed in 10 patients with Cushing's syndrome using a G.E. 8800 CT/T Body Scanner. The tomographic findings of unilateral adrenal masses in 4 patients were confirmed by surgery. In the remaining 6 patients, computerized tomography did not identify any masses, and both glands were well visualized and showed normal shape. In these patients, measurement of the adrenal glands revealed both normal (n=1) and enlarged glands (n=5). The findings of adrenalectomy (n=4) or transphenoidal surgery and follow-up (n=2) established the diagnosis of adrenal phyperplasia in all patients. (Author).

Congenital Intrahepatic Portosystemic Shunts

PMID:20812517

2009-12-01

Congenital Inner Ear Malformation: Three Dimensional Volume Rendering Image Using MR CISS Sequence

Intrahepatic portosystemic shunts are an anomalous connection between the portal vein and hepatic vein/IVC, which may be either congenital or acquired secondary to liver cirrhosis or portal hypertension. Cases of congenital intrahepatic shunts are usually encountered in children and may spontaneously resolve. We report 5 cases of congenital intrahepatic portosystemic shunts in neonates and an adult

2008-12-15

Structural and functional aspects of radiation adrenalopathy

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2003-10-01

Investigation and management of adrenal disease

As part of a combined study of the responses of the mouse adrenal gland and kidney to X-irradiation, the weight of adrenal glands, the relative volumes of the cortex and medulla and the aldosterone output were measured at predetermined times after irradiation of the left organs only.

1986-01-01

British Library Electronic Table of Contents (United Kingdom)

Disorders of the adrenal gland are rare and complex, with many potential pitfalls in their management. An understanding of embryology, anatomy, physiology and biochemistry is crucial. Surgical treatment may be required for syndromes of hormonal excess and/or suspicion of neoplasia.

2011-01-01

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Effect of capsaicin-sensitive sensory nerves on plasma glucose and catecholamine levels during 2-deoxyglucose-induced stress in conscious rats.

Moving toward personalized cell-based interventions for adrenal cortical disorders: Part 2 - Human diseases and tissue engineering

1. Sensory fibres innervate the adrenal medulla but their function is not known. In this paper we have studied the effect of capsaicin-sensitive sensory fibres on the adrenal catecholamine (CA) response...Full Text Available

1990-07-01

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Transdifferentiation of an individual's own cells into functional differentiated cells to replace an organ's lost function would be a personalized approach to therapeutics. In this two part series, we will describe the progress toward establishing functional transdifferentiated adrenal cortical cells. In this article (Part 2), we describe the disorders of the adrenal cortex, therefore establishing why there is the need for personalized cell-based therapy for individuals with these disorders. We then present our pilot studies of cell transdifferentiation toward an adrenal cortical fate using genes described in the first article of this pair (Part 1).

2011-01-01

Moving toward personalized cell-based interventions for adrenal cortical disorders: Part 1 - Adrenal development and function, and roles of transcription factors and signaling proteins

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Transdifferentiation of an individual's own cells into functional differentiated cells to replace an organ's lost function would be a personalized approach to therapeutics. In this two part series, we will describe the progress toward establishing functional transdifferentiated adrenal cortical cells. In this article (Part 1), we describe adrenal development and function, and discuss genes involved in these processess and selected for use in our pilot studies of transdifferentiation that are presented in the second article (Part 2).

2011-01-01

Computerized tomography of the adrenal glands in the Cushing Syndrome propaedeutic

Ultrabiomicroscopic-Histopathologic Correlations in Individuals with Autosomal Dominant Congenital Microcoria: Three-Generation Family Report

Portuguese (Mar 1983). Brazil Furlanetto, RP Abucham Filho, J. Albertotti,

1982-10-24

Long term indwelling urethral catheterisation for congenital neuropathic bladder.

BackgroundCongenital microcoria (CMC) is due to a maldevelopment of the dilator pupillae muscle of the iris, with a pupil diameter of less than 2 mm. It is associated with juvenile...Full Text Available

Hirschsprung disease associated with polydactyly, unilateral renal agenesis, hypertelorism, and congenital deafness: a new autosomal recessive syndrome.

Long term urethral catheterisation remains an important and effective method of achieving dryness and maintaining renal function in children with congenital neuropathic bladders. Those most likely to...Full Text Available

1983-04-01

Genetic Heterogeneity in Severe Congenital Neutropenia: How Many Aberrant Pathways Can Kill a Neutrophil?

An association of Hirschsprung disease with polydactyly, unilateral renal agenesis, hypertelorism, and congenital deafness is described in sibs (brother and sister) of consanguineous parents. It is...Full Text Available

1988-03-01

Congenital woolly hair without P2RY5 mutation

Purpose of reviewSevere congenital neutropenia (SCN) is a primary immunodeficiency in which lack of neutrophils causes inadequate innate immune host response to bacterial...Full Text Available

2007-12-01

Congenital anal anomalies in two families with the Opitz G syndrome.

Congenital woolly hair is a disorder with structural defects of the hair shafts. Curled hairs are noticed at birth or soon after birth and often improve with age. Some cases of woolly hairs are associated...Full Text Available

2009-01-01

CYP1B1 mutations in Spanish patients with primary congenital glaucoma: phenotypic and functional variability

Five children from two families presented to a regional neonatal surgical unit between 1959 and 1984 with congenital anal anomalies and other malformations resulting from an autosomal dominant inherited...Full Text Available

1987-11-01

A late presenting congenital diaphragmatic hernia misdiagnosed as spontaneous pneumothorax

PurposeTo analyze the contributions of cytochrome P4501B1 (CYP1B1) mutations to primary congenital glaucoma (PCG) in Spanish patients.MethodsWe...Full Text Available

A Combination of Dual-mode 2,940 nm Er:YAG Laser Ablation with Surgical Excision for Treating Medium-sized Congenital Melanocytic Nevus

Congenital diaphragmatic hernia (CDH) is described as (1) failure of diaphragmatic closure at development, (2) presence of herniated abdominal contents into chest and (3) pulmonary hypoplasia. Usually,...Full Text Available

2010-09-01

Trends in Neuroendocrinology

BackgroundThere are various treatment options for congenital melanocytic nevus (CMN), including surgical excision, dermabrasions, curettage, laser treatment, chemical peels and cryosurgery....Full Text Available

2009-05-01

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The mammalian circadian timing system is organized into hierarchical structures with a central clock in the suprachiasmatic nucleus (SCN) and subsidiary peripheral oscillators. After the discovery of the local clockwork in peripheral organs and tissues, which have a molecular makeup similar to the central pacemaker SCN, uncovering the roles of the peripheral clock in the rhythmic physiology has been an emerging goal in chronobiology. Glucocorticoid (GC) is a multifunctional adrenal steroid hormone that shows a robust circadian rhythm. The daily GC rhythm has long been thought to be governed by the SCN via the hypothalamus-pituitary-adrenal neuroendocrine axis. Recent findings, however, indicate that multiple regulatory mechanisms, including the adrenal intrinsic mechanism by the peripheral...

2011-01-01

Response to CRH Infusion in Cocaine-Dependent Individuals

Influence of temperature on strength of cemented surrogate nitrate salt waste

ContextCorticotropin-releasing hormone (CRH), through the hypothalamic pituitary adrenal (HPA) axis and other brain stress systems, is involved in the emotional dysregulation...Full Text Available

2009-04-01

Influence of temperature on strength of cemented surrogate nitrate salt waste

The Rocky Flats Plant (RFP) generates large volumes of a low level aqueous waste stream high in nitrate salts. The aqueous waste is concentrated by evaporation and then mixed with Portland cement prior to transport and disposal. Planned process upgrades include a new horizontal thin film evaporator. Temperature of brine at discharge end of the new evaporator will be near boiling point. Introduction of hot water to cement can degrade the monolithic waste form. However, the RFP salt waste contains high concentrations of compounds known to retard hydration. This paper discusses impact of introducing high temperature waste to cement. The study evaluated three waste compositions: (1) highest probable nitrate composition, (2) highest probable chloride composition, and (3) current composition. Results showed that compressive strength of final waste form increased with brine temperature, and waste forms from brine at the boiling point exhibited a near ...

1993-01-01

Urethrorectal fistula in a horse.

1993-03-01

Thalassemia and Venous Thromboembolism

Anomalies of the urethra are uncommon. Urethrorectal fistula in horses has only been reported in foals and only in conjunction with other congenital anomalies. This report describes the diagnosis, surgical...Full Text Available

1999-02-01

Repair of a Complex Congenital Cardiac Defect

Although the life expectancy of thalassemia patients has markedly improved over the last few decades, patients still suffer from many complications of this congenital disease. The presence of a high...Full Text Available

Medline Plus

... stenosis. It looks from the transesophageal and the 3-D echo that most of the problem is really ... posterior leaflet, which you can see on the 3-D echo image is a major source. The anterior ...

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Prosthetic rehabilitation of hypophosphatasia: a case report

Congenital nephrogenic diabetes insipidus in a baby girl.

Hypophosphatasia is a congenital disease characterized by deficiency of serum and tissue non-specific alkaline phosphatase activity. The disease occurs due to mutations in the liver/bone/kidney alkaline...Full Text Available

Temporal patterns of protein phosphorylation after angiotensin II, A23187 and/or 12-O-tetradecanoylphorbol 13-acetate in adrenal glomerulosa cells.

A 6-week-old girl with fever, hypernatraemia, dehydration, and polyuria failed to concentrate urine in response to exogenous vasopressin administration. There was no family history of nephrogenic diabetes...Full Text Available

1978-11-01

Effect of polychlorinated biphenyls (Aroclor 1254) on rhythmic pituitary-adrenal function. [Rats

The temporal patterns of protein phosphorylation in the adrenal glomerulosa cell were analysed by two-dimensional electrophoresis after stimulation with 10 nM-angiotensin II or various agents [10 nM-12-O-tetradecanoylphorbol...Full Text Available

1986-09-15

Dexamethasone-suppression adrenal scintigraphy in hyperandrogenism: concise communication

Numerous studies have documented the general toxicity of the polychlorinated biphenyls (PCBs) and a sizeable literature has accumulated concerning the toxic effects of PCBs on a variety of homeostatic systems. However, current information regarding the effects of PCBs on rhythmic endocrine function is quite limited. Thus, the present study was undertaken to determine whether circadian periodicity in pituitary-adrenal function is affected by exposure to PCBs.

1983-09-01

Klippel-feil syndrome with situs inversus. A rare association

To assess the contribution of adrenal-derived androgens in women with hirsutism, adrenal scintigrams under dexamethasone suppression (DS) were performed on 35 women with increasing facial or body hair and irregular or absent menses. Based upon the DS regimen chosen (8 mg/d for 2 days or 4 md/d for 7 days before the injection of 6..beta..-(/sup 131/I)iodomethylnorcholesterol), three imaging patterns were identified. The first was the absence of uptake before 3 days (8-mg DS) or before 5 days (4-mg DS) after injection. This imaging pattern was seen in 17 of the 35 patients studied and was considered normal. The second pattern was bilateral uptake earlier than 3 days (8-mg DS regimen) or 5 days (4-mg DS) after injection. This was seen in 13 of the 35 patients and was interpreted as bilateral early visualization. Adrenal-vein catheterization performed on six patients with this pattern showed increased ...

1981-01-01

Ambient air pollution and congenital heart disease: a register-based study.

Klippel-Feil Syndrome (KFS) is a congenital anomaly characterized by a defect in the formation or segmentation of the cervical vertebrae. The clinical triad consists of short neck, low posterior hairline and limited neck movement. Multiple congenital anomalies have been associated with this disease. This is a case of KFS in a young girl along with situs inversus, which is an extremely rare association. Various systemic associations occurring in this multi-system disorder are also discussed. (author)

2008-04-01

The Adrenal Mass: Correlation of Histopathology with Imaging

Maternal exposure to ambient air pollution has increasingly been linked to adverse pregnancy outcomes. The evidence linking this exposure to congenital anomalies is still limited and controversial. This case-control study investigated the association between maternal exposure to ambient particulate matter with aerodynamic diameter less than 10 ?m (PM(10)), sulfur dioxide (SO(2)), nitrogen dioxide, nitric oxide (NO), ozone (O(3)), and carbon monoxide (CO) and the occurrence of congenital heart disease in the population of Northeast England (1993-2003). Each case and control was assigned weekly average (weeks 3-8 of pregnancy) of pollutant levels measured by the closest monitor to the mother's residential postcode. Using exposure as both continuous and categorical variables, logistic regression models were constructed to quantify the adjusted odds ratios of exposure to air pollutants and the occurrence of each outcome group. We found exposure to ...

2011-02-17

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Background Computed tomography (CT) and magnetic resonance (MR) imaging can help diagnose benign adrenal adenomas, but prior studies rely on nonoperative follow-up as proof of a lesion?s benign nature. We examined adrenalectomy tissues to determine if imaging characteristics correlate with histopathologic findings. Methods We retrieved data for 196 consecutive adrenalectomies in 192 patients from 2000 to 2008. Imaging results were considered to signify benign adrenal adenoma if one or more of the following was present: Hounsfield units 60% or relative contrast washout of >40%, or MR with chemical-shift imaging demonstrating loss of signal intensity on out-of-phase images. Results The sensitivity and specif...

2010-01-01

Laparoscopic adrenal-sparing surgery: personal experience, review on technical aspects

British Library Electronic Table of Contents (United Kingdom)

Partial adrenalectomy is usually performed for the treatment of bilateral pheochromocytomas and in case of sporadic, monolateral tumors, to minimize the risk of adrenal failure, especially in younger patients. Due to the lack of consistent series, many issues such as correct surgical indications and technical aspects still need to be debated. From 2007 to 2010 we performed four unilateral partial adrenalectomies (3 aldosterone-producing adenomas and 1 cortisol-producing adenoma), and three bilateral subtotal adrenalectomies, consisting in total adrenalectomy on one side and partial adrenalectomy on the contralateral gland (3 bilateral pheochromocytomas in MEN IIa). In case of single tumor, partial adrenalectomy was carried out without adrenal vein ligation and the results were similar to t...

2011-01-01

Adrenal incidentaloma: Does an adequate workup rule out surprises?

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Background Adrenal incidentaloma remains a diagnostic challenge. Despite well-established management guidelines, the long-term results of following these guidelines are unknown. We sought to determine how accurately these guidelines identify functioning incidentalomas and how often these guidelines result in adrenalectomy for benign tumors. Methods We catalogued adrenal incidentalomas from a retrospective review of 500 consecutive adrenalectomies at a single institution. The outcome measures studied were patient demographics, preoperative biochemical analysis, imaging characteristics, tumor size, type of operation performed, and postoperative histologic diagnosis. Results Eighty-one of the 500 adrenalectomies performed were for incidentalomas. Size was the only significant characteristic t...

2010-01-01

Steroid hormones and brain development: some guidelines for understanding actions of pseudohormones and other toxic agents.

DIFFERENTIAL EXPRESSION OF HPA AXIS HOMOLOG IN THE SKIN

Gonadal, adrenal, and thyroid hormones affect the brain directly, and the sensitivity to hormones begins in embryonic life with the appearance of hormone receptor sites in discrete populations of neurons....Full Text Available

1987-10-01

Capsaicin-sensitive sensory neurons are involved in the plasma catecholamine response of rats to selective stressors.

SummaryHuman skin expresses elements of the hypothalamo-pituitary-adrenal (HPA) axis including pro-opiomelanocortin (POMC), corticotropin releasing hormone (CRH), the CRH receptor-1...Full Text Available

2007-02-01

Calcium gradients and buffers in bovine chromaffin cells.

1. The effect of capsaicin pre-treatment on adrenal catecholamine (CA) secretion in response to stress is controversial. In earlier experiments performed under pentobarbitone anaesthesia, the release...Full Text Available

1991-02-01

Adrenocortical (dys)function in septic shock - A sick euadrenal state

1. Digital imaging and photometry were used in conjunction with the fluorescent Ca2+ indicator, Fura-2, to examine intracellular Ca2+ signals produced by depolarization of single adrenal chromaffin...Full Text Available

1992-05-01

Medline Plus

... York, 7/15/2008) Cancers Adrenal Gland Cancer Laparoscopic Adrenalectomy (Shawnee Mission Medical Center, Shawnee Mission, KS, ... MN, 1/24/2007) Colorectal Cancer Advances in Laparoscopic Colorectal Surgery (Charles E. Schmidt College of Biomedical ...

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A central feature of the endocrine pathophysiology of septic shock is thought to be the existence of adrenal dysfunction. Based on changes in glucocorticoid secretion and responsiveness, protein binding, and activity. These changes have been described by the terms "Relative Adrenal Insufficiency" (RAI), or "Critical Illness Related Corticosteroid Insufficiency" (CIRCI), and form part of the rationale for trials of glucocorticoid treatment in septic shock. Diagnostic criteria for these conditions have been based on plasma cortisol profiles and have proven notoriously difficult to establish. The uncertainty in this area arises from the inability of current tests to clearly identify who is truly glucocorticoid "deficient" at a cellular level, and hence who requires supplemental glucocorticoid...

2011-01-01

The value of cardiac catheterization and cineangiography in infantile lobar emphysema

Extension of adrenal tumors into the vena cava: results of magnetic resonance angiography; Kavazapfen bei Nebennierentumoren: Kernspintomographische Befunde

Lobar emphysema is an uncommon cause of respiratory distress in infancy. Congenital heart disease is seen in about 20% of the patients with infantile (congenital) lobar emphysema. We described six infants with lobar emphysema. In three of them a congenital heart disease was demonstrated by cardiac catheterization and cineangiography; two had a tetralogy of Fallot with right aortic arch and the third infant a ventricular septal defect. The pulmonary angiography showed stretching of the arteries with very poor filling of the peripheral arteries and a characteristic smaller pulmonary vein in the affected lobe. In all the six patients the pulmonary artery pressure was normal. All the patients underwent lobectomy with good results. We feel that a preoperative cardiac catheterization and cineangiography is of value in this very sick group of infants. (orig.).

DEGRADED TBP SOLVENT REGENERATION TECHNOLOGY USING BUTYLAMINE AS A SOLVENT WASHING TO REDUCE SOLID SALT WASTE

In rare cases, large adrenal masses with a suspicion of malignancy exhibit tumor extension into the adrenal vein and inferior vena cava. When planning surgery, the relationship of the extension to the inferior vena cava is of decisive improtance. We describe in two cases on the detection of a tumor thrombus in the inferior vena cava arising from a malignant adrenal mass by means of MR angiography (TOF, coronary 2D GRE images [FLASH], 3 slices acquired during 15 second apnea). The angiograms correlated well with the results of DSA venacavography and with the intraoperative findings. Thus, MRA has been demonstrated to be suitable for the certain proof of a venous tumor thrombus not only in cases of renal cell carcinomas but also in cases of malignant adrenal masses. The method should be applied whenever there is evidence of a venous involvement in the adrenal MR images. (orig.) ...

1995-11-01

Y-type urethral duplication presented with perineal fistula in a boy

Normal butylamine compounds are studied as salt-free wash reagents for degraded solvent used in PUREX process in spent fuel reprocessing. The solvent wash tests were carried out with two types of butylamine compounds, n-butylamine oxalate and n-butylamine bicarbonate, by counter-current mode using a small size mixer-settler composed of two 4-stage wash steps. Di-n-butyl phosphoric acid (HDBP), the main degradation product from TBP, was removed from real degraded solvent with decontamination factor of 2.5 {approx} 7.9. The study on electrolytic decomposition of butylamine compounds was also conducted for waste treatment.

2003-02-27

Safety and efficacy of botox injection in alleviating post-operative pain and improving quality of life in lower extremity limb lengthening and deformity correction

Urethral duplication is a rare congenital anomaly of the lower urinary system and has varied presentation. According to the Effmann classification, type IIA2-Y urethral duplication is charcterized by...Full Text Available

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Role of serum carrier proteins in the peripheral metabolism and tissue distribution of thyroid hormones in familial dysalbuminemic hyperthyroxinemia and congenital elevation of thyroxine-binding globulin.

BackgroundDistraction osteogenesis is the standard treatment for the management of lower limb length discrepancy of more than 3 cm and bone loss secondary to congenital anomalies,...Full Text Available

Radionuclide angiocardiography in the diagnosis of congenital heart disorders.

To investigate the role of thyroxine-binding globulin (TBG) and albumin in the availability of thyroid hormones to peripheral tissues, comprehensive kinetic studies of thyroxine (T4) and triiodothyronine...Full Text Available

1987-08-01

Prosthodontic Rehabilitation Alternative of Patients with Cleft Lip and Palate (CLP): Two Cases Report

Radionuclide angiocardiography provides a noninvasive assessment of cardiac function and blood flow through the heart and lungs. During the past three years, this procedure has been used at the Duke...Full Text Available

1981-06-01

Poland's anomaly. Natural history and long-term results of chest wall reconstruction in 33 patients.

Although patients with cleft lip and palate (CLP) are not seen regularly in general dental practice, this is a frequent congenital anomaly; approximately one in every 800 live births results in a CLP....Full Text Available

2009-01-01

Phenotypic Transition of the Collecting Duct Epithelium in Congenital Urinary Tract Obstruction

Poland's anomaly is an uncommon congenital aberration of the chest wall characterized by absence of the pectoralis major muscle and other nearby musculoskeletal components. In this series, a wide spectrum...Full Text Available

1988-12-01

Pericardiectomy for Pleuropericardial Effusion Complicating Bacterial Pneumonia

Epithelial-mesenchymal transition (EMT) has emerged in recent years as an important process in the development of organ fibrosis in many human diseases. Our previous experience in a nonhuman primate...Full Text Available

2010-01-01

Severe pericardial effusion is a rare complication of bacterial pneumonia and it usually disappears under medical treatment. Herein we report a case of a girl with a congenital immunodeficient syndrome...Full Text Available

2010-01-01

Patent arterial duct

Multiple neural tube defects in the same patient with no neurological deficit

Patent arterial duct (PAD) is a congenital heart abnormality defined as persistent patency in term infants older than three months. Isolated PAD is found in around 1 in 2000 full term infants. A higher...Full Text Available

MRI of congenital heart disease in childhood

Congenital deformities involving the coverings of the nervous system are called neural tube defects (NTDs). NTD can be classified as neurulation defects, which occur by stage 12, and postneurulation...Full Text Available

2010-01-01

Loss of lysophosphatidylcholine acyltransferase 1 leads to photoreceptor degeneration in rd11 mice

MRI provides a non-invasive diagnostic tool complementing echocardiography on one hand, and showing advantages over echocardiography, on the other hand, especially after corrective procedures. The multiple different MRI sequences need to be adapted to examinations of children and patients with congenital heart disease (CHD), and can be used to detect morphologic changes, blood-flow in the heart and thoracic vessels and diastolic or systolic function of myocardium. Several factors determine the success of the examination of a complex congenital heart disease or a postoperative situs. Pediatric radiologists and radiologists experienced in congenital heart diseases have to work in close cooperation. Echocardiography should be performed before MRI. The results of prior examinations and the clinical history of the patients, including possible palliative or reconstructive operations, must be available before MRI to guide the ...

2004-02-01

Intrauterine tracheal obstruction, a new treatment for congenital diaphragmatic hernia, decreases amniotic fluid sodium and chloride concentrations in the fetal lamb.

Retinal degenerative diseases, such as retinitis pigmentosa and Leber congenital amaurosis, are a leading cause of untreatable blindness with substantive impact on the quality of life of affected individuals...Full Text Available

2010-08-31

Hypothalamo-pituitary hormone insufficiency associated with cleft lip and palate.

OBJECTIVE: To evaluate the effect of fetal tracheal occlusion on sodium and chloride concentrations in amniotic and tracheal fluid. SUMMARY BACKGROUND DATA: Intrauterine tracheal occlusion has been...Full Text Available

1997-12-01

Functional and Behavioral Restoration of Vision by Gene Therapy in the Guanylate Cyclase-1 (GC1) Knockout Mouse

Two male patients with congenital cleft lip and palate first seen at ages 10.2 and 21.5 years presented with typical signs of hypothalamic-interior pituitary hormone deficiencies. They were found to...Full Text Available

1978-12-01

Fetal, perinatal, and infant death with congenital renal anomaly

BackgroundRecessive mutations in guanylate cyclase-1 (Gucy2d) are associated with severe, early onset Leber congenital amaurosis-1(LCA1). Gucy2d...Full Text Available

Cystic Dysplasia of the Rete Testis Accompanying an Inguinal Hernia: A 63-Year-Old Man

Aims: To ascertain why 19.6% of pregnancies in which a fetal renal anomaly has been detected fail to produce a surviving child, and whether antenatal diagnostic accuracy has altered since...Full Text Available

2002-08-01

Comprehensive SNP-chip for retinitis pigmentosa-Leber congenital amaurosis diagnosis: new mutations and detection of mutational founder effects

Cystic dysplasia of the rete testis (CDT) is a very rare congenital benign testicular tumor that is often associated with ipsilateral genitourinary anomalies. It is usually found in the pediatric population...Full Text Available

2011-07-01

Complex Integration of Matrix, Oxidative Stress, and Apoptosis in Genetic Emphysema

Fast and efficient high-throughput techniques are essential for the molecular diagnosis of highly heterogeneous hereditary diseases, such as retinitis pigmentosa (RP). We had previously approached RP...Full Text Available

2010-01-01

Screening for congenital hypothyroidism in Lebanon - An example of newborn screening in absence of mandatory law

Alveolar enlargement, which is characteristic of bronchopulmonary dysplasia, congenital matrix disorders, and cigarette smoke-induced emphysema, is thought to result from enhanced inflammation and ensuing...Full Text Available

2009-07-01

Retroperitoneal endoscopic adrenalectomy is safe and effective

This paper presents a group's efforts to introduce national newborn screening in Lebanon. Tandem mass spectrometry (MS) technology offers the prospect of utilizing filter paper blood specimens to screen newborns for several metabolic disorders. In order to be cost-effective, it is necessary to have a mandatory newborn screening policy. At present such a policy is not available in Lebanon. A national screening survey for congenital hypothyroidism (CH) was carried out to assess the incidence of this anomaly in the Lebanese population. Such an initiative is essential as a first step for assessing the feasibility and cost of a national screening programme. Coated beads, blood spot standards for quality control and buffer solutions used for assays were prepared in-house to minimize costs. At first, samples of umbilical cord blood spots were assayed for TSH using IRMA technique. Those samples showing borderline or high values of TSH were then assayed for total T4 (TT4) ...

2004-10-01

British Library Electronic Table of Contents (United Kingdom)

AbstractBackground: The aim of this study was to review an experience with retroperitoneal endoscopic adrenalectomy (REA). This is the procedure of choice for adrenal tumours at this institution. Methods: Between 1997 and 2008, 112 REAs were performed in a single university centre. Data were retrieved retrospectively from a prospectively collected database, including information on patient demographics, surgical procedure, complications and hospital stay. Results: One hundred and twelve REAs were carried out successfully in 105 patients, including seven bilateral adrenalectomies. Thirty nine patients with unilateral adrenal disease had a phaeochromocytoma, of whom 16 had multiple endocrine neoplasia syndrome type 2, 21 patients had Cushing's disease and 20 had Conn's disease. Median body m...

2010-01-01

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Prognostic factors for survival after surgery for adrenal metastasis

British Library Electronic Table of Contents (United Kingdom)

Aim To better define the indications for adrenalectomy for adrenal metastasis we have analysed factors predicting survival in our institutional series. Methods A consecutive series of 30 patients undergoing adrenalectomy for metastasis (1996-2007), excluding patients with simultaneous ipsilateral renal cell carcinoma (RCC), was studied. Metastases were regarded as synchronous (6 mo), depending on the interval after primary surgery. Survival was calculated from time of adrenalectomy and factors influencing survival were identified. Results The tumour diagnoses were RCC n = 9, malignant melanoma n = 5, non-small-cell lung cancer n = 5, colorectal carcinoma n = 4, foregut carcinoid n = 2, adrenocortical carcinoma, breast cancer, hepatocellular carcinoma, urothelial c...

2010-01-01

Exploration of the hypothalamic?pituitary?adrenal function as a tool to evaluate animal welfare

British Library Electronic Table of Contents (United Kingdom)

Measuring HPA axis activity is the standard approach to the study of stress and welfare in farm animals. Although the reference technique is the use of blood plasma to measure glucocorticoid hormones (cortisol or corticosterone), several alternative methods such as the measurement of corticosteroids in saliva, urine or faeces have been developed to overcome the stress induced by blood sampling itself. In chronic stress situations, as is frequently the case in studies about farm animal welfare, hormonal secretions are usually unchanged but dynamic testing allows the demonstration of functional changes at several levels of the system, including the sensitization of the adrenal cortex to ACTH and the resistance of the axis to feedback inhibition by corticosteroids (dexamethasone suppression t...

2007-01-01

Effect of aspirin on hypothalamic?pituitary?adrenal function and on neuropsychological performance in healthy adults: a pilot study

British Library Electronic Table of Contents (United Kingdom)

Rationale Hypothalamic?pituitary?adrenal axis dysregulation predicts poor clinical and biochemical response to antidepressants. Antiglucocorticoids have therapeutic benefits but most have a troublesome adverse event profile. Aspects of neuropsychological performance, notably working memory, are susceptible to corticosteroid modulation and are impaired in depression. Aspirin has been shown to attenuate the adrenocorticotropic hormone (ACTH) and cortisol response to physiological challenge suggesting its potential to act as an augmenting agent in depression. Objectives To examine the effect of sub-acute (300?mg daily for 7?days) aspirin pre-treatment on the cortisol awakening response and the effect of acute (600?mg) and sub-acute aspirin on the neuroendocrine and neuropsychological response...

2009-01-01

Chronic maternal stress affects growth, behaviour and hypothalamo?pituitary?adrenal function in juvenile offspring

British Library Electronic Table of Contents (United Kingdom)

Maternal stress during pregnancy, particularly that combined with low socioeconomic status (SES), has been linked to an increased risk for impaired behavioural and emotional development and affective disorders in children. In animal models, acute periods of prenatal stress have profound effects on hypothalamo-pituitary-adrenal (HPA) function and behaviour. However, few studies have determined the impact of chronic exposure to stress in animal models. The objective of this study was to determine the effects of chronic maternal stress (CMS) during the 2nd half of pregnancy and nursing on growth, locomotor behaviour and HPA axis function in juvenile guinea pig offspring. Pregnant guinea pigs were exposed to a random combination of variable stressors every other day over the 2nd half of gestat...

2008-01-01

Solidification of DOE problem wastes

Los Alamos second-generation system for passive and active neutron assays of drum-size containers

Sodium nitrate waste has been successfully solidified in two types of polymeric materials: polyethylene, a thermoplastic material, and polyester styrene (PES), a thermosetting material. Waste form property evaluation tests such as ANS 16.1 leaching test and compressive strength measurements were performed on the waste forms containing various amounts of sodium nitrate. A single-screw extruder was employed for incorporating dry waste into polyethylene at its melt temperature of 120/sup 0/C to produce a homogenous mixture. Results of the leaching test for polyethylene waste forms containing 30, 50, 60 and 70 wt% sodium nitrate are presented as cumulative fraction leached and leaching indices ranging from 11 to 7.8. Two PES systems are discussed. The first is for solidification of dry salt wastes and the second is a water extendible system that is compatible with wet waste streams. Leaching data for PES and water extendible PES waste forms ...

1986-01-01

The chance finding of an aneurysm of the right sinus of Valsalva in an 11-year-old child with a ventricular septal defect and a pericardial effusion

We describe in a comprehensive fashion the Los Alamos second-generation system for passive and active neutron assays of drum-size containers. The developmental history of this 7-year project is presented with emphasis on the pulsed active neutron technique (differential dieaway), which has achieved milligram levels of assay sensitivity for both plutonium and uranium wastes. We describe in detail the matrix effects for both passive and active neutron assays. We present in a thorough fashion our novel approach to achieving comprehensive corrections for these matrix effects using measurements made during the assays. We develop a matrix correction formalism based on separate neutron absorption and moderator indices determined from these measurements. These are presented as a series of analytic functions fitted to the data. Absolute calibrations and calibration standards are discussed, as is a practical means (pink drum measurements) of achieving routine calibration verification at all ...

1972-09-17

British Library Electronic Table of Contents (United Kingdom)

Ventricular septal defects can occur as part of other congenital cardiac malformations or as an isolated finding. Aneurysms of the sinus of Valsalva are rare, most commonly involving the right or noncoronary sinuses. They can be congenital or acquired through infection, trauma, or degenerative diseases. They frequently co-exist with ventricular septal defects, aortic valve dysfunction, or other cardiac abnormalities. More commonly, sinus of Valsalva aneurysms are diagnosed after the clinical sequelae of rupture. Several etiologic factors may lead to the development of pathologic pericardial effusion and the detection of pericardial effusion was one of the first applications of echocardiography to gain widespread acceptance. We present a case of a chance finding of an aneurysm of the right ...

2011-01-01

Severe immune haemolytic anaemia due to ceftriaxone in a patient with congenital nephrotic syndrome

British Library Electronic Table of Contents (United Kingdom)

Abstract Aim:- To describe the first case of ceftriaxone-related haemolysis in a patient with congenital nephrotic syndrome (CNS). Background:- Severe haemolysis caused by an immune reaction to ceftriaxone has mostly been described in patients with underlying haematological or immune dysfunction. Case report:- The authors present a 20-month-old boy with CNS of the Finnish type with several previous severe infections treated with ceftriaxone, admitted for suspected sepsis. Following ceftriaxone administration he developed shock secondary to an acute haemolytic reaction, with severe anaemia. Hypersensitivity to ceftriaxone was documented through positive agglutination tests. Conclusion:- Onset of haemolysis following ceftriaxone administration, particularly in a patient previously exposed to...

2011-01-01

Endoscopic management of congenital esophageal stenosis

British Library Electronic Table of Contents (United Kingdom)

Background/Purpose: Congenital esophageal stenosis (CES) is a rare malformation. Endoscopic dilations represent a therapeutic option. This study retrospectively evaluated the efficacy and safety of a conservative treatment of CES. Patients and Methods: Patients diagnosed with CES since 1980 by a barium study or endoscopy were reviewed. Endoscopic ultrasonography (Olympus UM-3R-20-MHz radial miniprobe, Olympus Corporation, Tokyo, Japan), available from 2001, allowed for the differential diagnosis of tracheobronchial remnants (TBR) and fibromuscular hypertrophy (FMH) CES. All children underwent conservative treatment by endoscopic dilations (hydrostatic and Savary). Results: Forty-seven patients (20 men) had CES. Fifteen were associated with esophageal atresia; and 8, with Down syndrome. Mea...

2011-01-01

Selective stimulation of catecholamine release from bovine adrenal chromaffin cells by an ionotropic purinergic receptor sensitive to 2-methylthio ATP

Bilateral adrenocortical carcinoma in a patient with multiple endocrine neoplasia type 1 (MEN1) and a novel mutation in the MEN1 gene

Background2-Methylthioadenosine 5'-triphosphate (2-MeSATP), formerly regarded as a specific P2Y (metabotropic) purinergic receptor agonist, stimulates Ca²⁺influx and...Full Text Available

Atlas of nuclear medicine imaging

The incidence of adrenal involvement in MEN1 syndrome has been reported between 9 and 45%, while the incidence of adrenocortical carcinoma (ACC) in MEN1 patients has been reported between 2.6 and 6%....Full Text Available

Separation and Measurement of Plant Alkaloid Enantiomers by RP-HPLC Analysis of their Fmoc-Alanine Analogs

An exhaustive compendium of normal and abnormal imaging is presented. The following systems and procedures are illustrated by over 2800 captioned illustrations: liver, spleen, and hepatobiliary imaging; perfusion and ventilation lung imaging; thyroid imaging; salivary gland imaging; bone imaging; brain imaging; radionuclide cisternography; blood flow studies; gallium imaging; nuclear cardiology; and renal imaging. Not included in the atlas are adrenal imaging procedures and emission computed tomography. (ERB)

1981-01-01

Screening for congenital hypothyroidism in Lebanon

Plants often synthesize secondary metabolites that are enantiomers. Enantiomers can cause very different physiological responses. Ammodendrine (1) and anabasine (2) are teratogens that can cause congenital malformations in livestock and enantiomeric forms of each have been found in Lupinus spp. an...

Practical approach to the loss of smell

Full text.National programs of neonatal screening were introduced in most countries and have proved their effectiveness against different types of retardation in children. Congenital hypothyroidism is one of the most common causes of physical and mental retardation in children. Programs of screening have shown that the incidence of this disorder is ethnic-background: it is low in Africans: 1-32000 whereas it is 1-3500 and 1-5000 in Hispanic and European newborns respectively. No previous mass screening programs were done in Lebanon. For this purpose, this program was introduced in collaboration with the IAEA. Cord blood is spotted into circles on filter paper just after delivery. These samples are air-dried at room temperature and kept at 4 degree C. They are then collected from hospitals to be screened. In the laboratory, all samples are assayed for IRMA TSH first. Those showing high or borderline TSH level (20mU/L) are then assayed for RIA TT4. Only those showing ...

1999-11-02

Isolated dextrogastria: report of two cases

Loss of the sense of smell can be easily confirmed in any physician's office by having the patient try to identify various odors. The etiology of anosmia can be extremely varied, including nasopharyngeal disorders such as rhinitis and tumors; neurologic conditions such as head trauma, neoplasms, vascular lesions and infections of the central nervous system; viral infections; familial and congenital disorders; drugs; industrial exposure; endocrine diseases, and several other disorders. The prognosis of anosmia is guarded, and its treatment depends on the etiology.

1982-09-01

Further means for reducing radiation dose during exposure of the pelvis in infants

Isolated situs inversus of the stomach with otherwise normal position of the thoracic and abdominal viscera is an extremely rare anomaly occurring in two distinct forms. Because of its close relationship to the liver, the right-sided stomach may produce interesting and confusing changes in the liver scan. Two cases illustrate the radiologic features of the two principal types of isolated congenital dextrogastria and the effect of one type on the liver scan.

Distribution of /sup 14/C after oral administration of (1-/sup 14/C)linoleic acid in rats fed different levels of essential fatty acids

A simple dose-saving procedure for the diagnosis of congenital hip dysplasia employing a diaphragm in nearfocus position is presented. Doses to the skin and the gonads were measured with a phantom for the new and other techniques. With optimal techniques the skin dose is smaller than 2 mR and the gonadal doses lie around 0.1 mR for both male and female patients. An estimate of the dose burden from undesired radiation is appended. (orig.).

Cocaine in pregnancy: the effect of maternal administration of cocaine on the maternal and fetal pituitary-adrenal axes.

Rats from an inbred Sprague-Dawley strain were fed semisynthetic diets with a low (0.3 energy percent (en %)), normal (3 en %) or high (10 en %) content of essential fatty acids (EFA) for at least three generations. Twenty-nine- to 33-day-old male rats were given a single intragastric dose of (1-14C)linoleic acid in olive oil, and the respiratory CO2, urine and feces were collected for 46 hours (expt 1) or 20 hours (expt 2). The 14C activity in respiratory CO2, feces, urine and the carcass was determined in both experiments. In experiment 2 it was also measured in samples of the brown fat, liver, adrenals, white fat, skeletal muscles and brain. In both experiments the rats fed the low EFA diet retained significantly more 14C activity than the rats fed the normal or high EFA diets. In all groups the concentration of label was highest in the brown fat and the adrenals, but the above differences among the groups with respect to 14C retention were ...

1984-09-01

Accumulation and distribution of "8"5Kr in rats exposed to "8"5Kr atmospheres

The effects of cocaine on the maternal and fetal pituitary-adrenal axis in vivo during pregnancy have not been reported. Six pregnant ewes and their fetuses underwent instrumentation at 113 to 119 days' gestation. Ewes were assigned to receive an intravenous bolus injection of vehicle or cocaine (0.5, 1.0, or 2.0 mg.kg-1) at 124 to 136 days' gestation. Maternal arterial blood gases, fetal pH and fetal PCO2 were unchanged after injection of cocaine or vehicle. After administration of 2.0 mg.kg-1 cocaine, arterial fetal PO2 fell 3.2 +/- 1.72 mm Hg (p less than 0.05) at +5 minutes, returning to baseline by +15 minutes. Maternal and fetal adrenocorticotropin levels rose within 5 minutes after the highest cocaine dose (p less than 0.05). There was a significant (p less than 0.05) increase in maternal cortisol at all doses of cocaine and in fetal cortisol at +15 minutes after the 2.0 mg.kg-1. PMID:1847006

1991-02-01

100

Molecular aspects involved in swimming exercise training reducing anhedonia in a rat model of depression.

Retention kinetics and tissue partition coefficients for "8"5Kr were determined in rats exposed to known concentrations of "8"5Kr gas. The tissue partition coefficients were used to calculate the relative tissue radiation dose, assuming exposure conditions equivalent to the maximum permissible concentration in air (MPC)a. The estimated radiation dose was highest in adrenals and body fat, about twice the dose estimated for the next highest tissue, ovaries. Saturation and desaturation curves show that the contents of the large intestines have a prolonged desaturation time compared to other samples analyzed.

1977-05-01

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101

'Hair-on-end' skull changes resembling thalassemia caused by marrow expansion in uncorrected complex cyanotic heart disease

Patients suffering from depression frequently display hyperactivity of the hypothalamic-pituitary-adrenal axis (HPA) resulting in elevated cortisol levels. One main symptom of this condition is anhedonia. There is evidence that exercise training can be used as a rehabilitative intervention in the treatment of depressive disorders. In this scenario, the aim of the present study was to assess the effect of an aerobic exercise training protocol on the depressive-like behavior, anhedonia, induced by repeated dexamethasone administration. The study was carried out on adult male Wistar rats randomly divided into four groups: the "control group" (C), "exercise group" (E), "dexamethasone group" (D) and the "dexamethasone plus exercise group" (DE). The exercise training consisted of swimming (1 h/d, 5 d/wk) for 3 weeks, with an overload of 5% of the rat body weight. Every day rats were injected with either dexamethasone (D/DE) or saline solution (C/E). Proper positive ...

2011-06-15

102

Quarterly progress report for the Chemical and Energy Research Section of the Chemical Technology Division: October-December 1997

''Hair-on-end'' skull changes resembling thalassemia were rarely described in the 1950s and 1960s in children with cyanotic congenital heart diseases; these changes were described almost entirely in patients with tetralogy of Fallot or D-transposition of the great arteries. As these lesions have become correctable, the osseous changes, never common, seem now only to exist in a small number of patients with uncorrectable complex cyanotic congenital heart disease who survive in a chronic hypoxic state. We present two cases: a case of marked marrow expansion in the skull of a 5-year-old boy with uncorrectable cyanotic heart disease studied by CT, and a second case of an 8-year-old with tetralogy of Fallot and pulmonary atresia studied by plain skull radiographs. The true incidence of these findings is unknown. (orig.)

2005-07-01

103

Trends in adrenalectomy: a recent national review

This report summarizes the major activities conducted in the Chemical and Energy Research Section of the Chemical Technology Division at Oak Ridge National Laboratory (ORNL) during the period October--December 1997. The section conducts basic and applied research and development in chemical engineering, applied chemistry, and bioprocessing, with an emphasis on energy-driven technologies and advanced chemical separations for nuclear and waste applications. The report describes the various tasks performed within six major areas of research: Hot Cell Operations, Process Chemistry and Thermodynamics, Separations and Materials Synthesis, Fluid Structure and Properties, Biotechnology Research, and Molecular Studies. The name of a technical contact is included with each task described, and readers are encouraged to contact these individuals if they need additional information. Activities conducted within the area of Hot Cell Operations included efforts to optimize the processing conditions ...

1999-02-01

104

British Library Electronic Table of Contents (United Kingdom)

Background Adrenalectomy remains the definitive therapy for most adrenal neoplasms. Introduced in the 1990s, laparoscopic adrenalectomy is reported to have lower associated morbidity and mortality. This study aimed to evaluate national adrenalectomy trends, including major postoperative complications and perioperative mortality. Methods The Nationwide Inpatient Sample was queried to identify all adrenalectomies performed during 1998?2006. Univariate and multivariate logistic regression were performed, with adjustments for patient age, sex, comorbidities, indication, year of surgery, laparoscopy, hospital teaching status, and hospital volume. Annual incidence, major in-hospital postoperative complications, and in-hospital mortality were evaluated. Results Using weighted national estimate, 4...

2010-01-01

105

The use of steroids in the treatment of severe sepsis and septic shock

British Library Electronic Table of Contents (United Kingdom)

Sepsis and septic shock remain major causes of mortality and morbidity worldwide. Previously, high dose corticosteroids were used to dampen the inflammatory response but studies and meta-analyses showed this to be of no benefit and possibly detrimental. Subsequently, low dose corticosteroids were used in the treatment of sepsis and septic shock with the hypothesis that these conditions are associated with relative adrenal insufficiency. Although some studies showed promising results larger studies and meta-analyses have failed to reproduce these effects and the use of corticosteroids in the treatment of sepsis and septic shock remains controversial. We review the current literature and guidelines regarding low dose corticosteroid use in the management of sepsis and septic shock.

2011-01-01

106

New procedures. Comprehensive staging of lung cancer by MRI

Mode of action of membrane-disruptive lytic compounds from the marine dinoflagellate Alexandrium tamarense

Lung cancer staging according to the TNM system is based on morphological assessment of the primary cancer, lymph nodes and metastases. All aspects of this important oncological classification are measurable with MRI. Pulmonary nodules can be detected at the clinically relevant size of 4-5 mm in diameter. The extent of mediastinal, hilar and supraclavicular lymph node affection can be assessed at the same time. The predominant metastatic spread to the adrenal glands and spine can be detected in coronal orientation during dedicated MRI of the lungs. Search focused whole body MRI completes the staging. Various additional MR imaging techniques provide further functional and clinically relevant information during a single examination. In the oncological context the most important techniques are imaging of perfusion and tumor motion. Functional MRI of the lungs complements the pure staging and improves surgical approaches and radiotherapy planning. (orig.)

2010-08-01

107

British Library Electronic Table of Contents (United Kingdom)

Certain allelochemicals of the marine dinoflagellate Alexandrium tamarense cause lysis of a broad spectrum of target protist cells but the lytic mechanism is poorly defined. We first hypothesized that membrane sterols serve as molecular targets of these lytic compounds, and that differences in sterol composition among donor and target cells may cause insensitivity of Alexandrium and sensitivity of targets to lytic compounds. We investigated Ca^2^+ influx after application of lytic fractions to a model cell line PC12 derived from a pheochromocytoma of the rat adrenal medulla to establish how the lytic compounds affect ion flux associated with lysis of target membranes. The lytic compounds increased permeability of the cell membrane for Ca^2^+ ions even during blockade of Ca^2^+ channels wit...

2011-01-01

108

Laparoendoscopic single-site (LESS) retroperitoneal adrenalectomy using a homemade single-access platform and standard laparoscopic instruments

British Library Electronic Table of Contents (United Kingdom)

Background This study aimed to evaluate laparoendoscopic single-site (LESS) adrenalectomy via the retroperitoneal approach using the Alexis wound retractor with standard laparoscopic instrumentation. Methods Since October 2009, seven LESS retroperitoneal adrenalectomies have been completed successfully with a homemade single port created using an Alexis wound retractor as an access platform through a 3-cm incision beneath the tip of the 12th rib. Results All the LESS procedures for these seven patients with adrenal tumors (size, 1.3?6.0?cm; 4 right, 1 left) were completed successfully without traditional laparoscopic conversion or complication. The average operative time was 159?min, and the estimated blood loss was 100?ml. The average hospital stay was 2?days (range, 1?3?days). Conclusion...

2011-01-01

109

Biological effects of electromagnetic radiation in the microwave range

An animal model of social instability stress in adolescence and risk for drugs of abuse

The book examines current experimental and clinical knowledge concerning the biological and biophysical effects of electromagnetic radiation, particularly that in the microwave range. The biophysical bases of the interaction of electromagnetic radiation with matter are reviewed with emphasis on biological systems, and the effects of radiation on critical biological systems, including the nervous, reproductive, visual and blood-forming systems are compared. Data concerning the lethal effects of nonionizing radiation is presented and characteristics of the effects of electromagnetic radiation on the whole mammalian organisms are examined. Various reactions of the neuroendocrine system to electromagnetic radiation are described, with particular attention given to the adrenal system, and the combined effects of ionizing and microwave radiation in the flight environment are discussed. Results are also presented of a series of animal experiments on the quantitative ...

1980-01-01

110

British Library Electronic Table of Contents (United Kingdom)

There is increasing evidence that adolescence, like early life, is a sensitive period in which ongoing brain development can be influenced by environmental factors. This review describes our use of social instability as a model of mild adolescent social stress, its effects on social interactions and on hypothalamic?pituitary?adrenal function over the course of the procedure and in response to new stressors. The effects of social instability are sex-specific, with qualitative differences between the sexes on HPA function over the course of the stressor procedure, and with greater effects in males on behaviour observed during the social instability and greater effects in females on behavioural responses to drugs of abuse into adulthood, long after the stress exposure. The results from invest...

2010-01-01

111

Amphiphysin (Amph) maps to the proximal region of mouse chromosome 13

Medical management of motility disorders in patients with intestinal failure: a focus on necrotizing enterocolitis, gastroschisis, and intestinal atresia

Amphiphysin is a protein concentrated in neuronal synapses and peripherally associated with neurotransmitter vesicles. It is expressed in many neurons of the central and peripheral nervous systems, in the adrenal medulla, in the anterior and posterior pituitary, in cell lines of the endocrine pancreas, and in spermatocytes. Its subcellular localization and tissue distribution indicate a potential involvement in mechanisms of regulated exocytosis. A role in the dynamic organization of the membrane-associated cytoskeleton is suggested by structural homology to the products of two yeast genes, RVS161 and RVS167, whose mutation results in an abnormal actin distribution, disturbs budding morphology, and impairs cell entry into stationary phase. Limited stretches of sequence similarity, including an SH3 domain, are also shared with other actin-binding proteins. Amphiphysin is the dominant autoantigen in paraneoplastic Stiff-Man syndrome, a neurological autoimmune ...

1995-07-20

112

British Library Electronic Table of Contents (United Kingdom)

Background: Intestinal failure (IF) is the dependence upon parenteral nutrition to maintain minimal energy requirements for growth and development. It may occur secondary to a loss of bowel length, disorders of motility, or both. Short bowel syndrome (SBS) is a malabsorptive state resulting from surgical resection, congenital defect, or diseases associated with loss of absorptive surface area. A particularly vexing problem is associated with whole bowel and/or segmental intestinal dysmotility. Motility disorders within the context of SBS and IF may relate to rapid intestinal transit secondary to loss of intestinal length, dysmotility associated with loss or poor antegrade peristalsis, or gastroparesis. Therapy may be classified into medical (prokinetic and antidiarrheal agents) and surgica...

2011-01-01

113

MRI of congenital heart disease in childhood; MR-Tomographie kongenitaler Herzvitien im Kindesalter

KMeyeDB: a graphical database of mutations in genes that cause eye diseases

2004-02-01

114

British Library Electronic Table of Contents (United Kingdom)

KMeyeDB () is a database of human gene mutations that cause eye diseases. We have substantially enriched the amount of data in the database, which now contains information about the mutations of 167 human genes causing eye-related diseases including retinitis pigmentosa, cone-rod dystrophy, night blindness, Oguchi disease, Stargardt disease, macular degeneration, Leber congenital amaurosis, corneal dystrophy, cataract, glaucoma, retinoblastoma, Bardet-Biedl syndrome, and Usher syndrome. KMeyeDB is operated using the database software MutationView, which deals with various characters of mutations, gene structure, protein functional domains, and polymerase chain reaction (PCR) primers, as well as clinical data for each case. Users can access the database using an ordinary Internet browser wi...

2010-01-01

115

Infant well-being following neonatal cardiac surgery

British Library Electronic Table of Contents (United Kingdom)

Aims and objectives.- To investigate infant well-being as measured by feeding and sleeping and parental support following discharge from the NICU in infants following major cardiac surgery. Background.- Infant feeding and sleeping have been identified as two of the most important concerns reported by parents. These concerns have been reported anecdotally for infants who have undergone cardiac surgery in the neonatal period. Design.- A prospective study using questionnaires and phone interviews followed a cohort of parents of neonates who underwent surgery in the neonatal period for congenital heart disease. Methods.- The study was conducted using validated questionnaires and phone interviews with a semi-structured questionnaire. The questionnaires were administered prior to discharge from ...

2011-01-01

116

Familial unilateral deafness and delayed endolymphatic hydrops

British Library Electronic Table of Contents (United Kingdom)

Delayed endolymphatic hydrops (DEH) is a unique disorder characterized by fluctuating otologic symptoms in the setting of preexisting unilateral deafness. The symptoms include aural fullness, fluctuating hearing, and/or episodes of vertigo similar to those observed in Meniere disease and may occur ipsilateral or contralateral to the previously deafened ear. In most reported cases, the unilateral deafness has been a profound sensorineural hearing loss with a sudden onset that has been variously attributed to bacterial or viral labyrinthitis, acoustic or cranial trauma, otosclerosis, and congenital CMV infection. Familial occurrence of the syndrome has not previously been reported in the literature. In this report, we describe two possible familial instances of delayed DEH. These patients ra...

2007-01-01

117

Early-stage focal nodular hyperplasia: US/CT/MR features correlated with histology

Comparison of angiotensin II and SAR1, Ile8-AII binding to solubilized receptors in the bovine adrenal

Two cases of focal nodular hyperplasia (FNH), in which ultrasound (US), computed tomography (CT), and magnetic resonance imaging (MR) studies detected an atypical hemorrhagic pattern associated with an intrahepatic arterio venous malformation (AVM) around the growths, are presented. In both cases, histology demonstrated a very early regenerative stage and necrotic-hemorrhagic areas within the lesions. In these cases, the analysis of radiological findings, surgical specimens and histology seemed to confirm the pathogenetic hypothesis suggested by Wanless: in normal liver parenchyma, a ``blood steal`` phenomenon due to congenital or acquired intrahepatic AVM could cause ischemic damage, appearing as a hemorrhagic necrotic area, the extent of which depends on the degree of residual portal supply. (orig.)

1994-02-01

118

The neuroimmune-endocrine axis: pathophysiological implications for the central nervous system cytokines and hypothalamus-pituitary-adrenal hormone dynamics

Derivatives of angiotensin with a sarcosine substitution at the carboxy terminus and an aliphatic amino acid substitution at the amino terminus have been shown to be competitive ligands for the angiotensin receptor. Scatchard plots derived from saturation isotherm data in bovine adrenal particulates consistently demonstrate three to five times the number of binding sites for "1"2"5I-Sarl,Ile8-AII ("1"2"5I-SI-AII) than for "1"2"5I-AII in experiments in which the two ligands are run side-by-side. A significant difference between "1"2"5I-AII and "1"2"5I-SI-AII binding to detergent solubilized angiotensin receptors was observed. Of the "1"2"5I-AII binding sites, about 85-90% were recovered upon solubilization. In contrast, the recovery of "1"2"5I-SI-AII binding sites was only 4-5%. To determine whether binding sites labeled in the soluble state represent biologically significant AII receptors, they examined the ability of selected angiotensin analogs to inhibit ...

1986-04-13

119

Scientific Electronic Library Online (English)

Abstract in english Cytokines are molecules that were initially discovered in the immune system as mediators of communication between various types of immune cells. However, it soon became evident that cytokines exert profound effects on key functions of the central nervous system, such as food intake, fever, neuroendocrine regulation, long-term potentiation, and behavior. In the 80's and 90's our group and others discovered that the genes encoding various cytokines and their receptors are e (more) xpressed in vascular, glial, and neuronal structures of the adult brain. Most cytokines act through cell surface receptors that have one transmembrane domain and which transduce a signal through the JAK/STAT pathway. Of particular physiological and pathophysiological relevance is the fact that cytokines are potent regulators of hypothalamic neuropeptidergic systems that maintain neuroendocrine homeostasis and which regulate the body's response to stress. The mechanisms by which cytokine ...

2000-10-01

120

Studies on the biological effects of chemical defense mechanisms in vivo activated by low-dose irradiation

Salivary cortisol assessment in the evaluation of hypothalamic-pituitary-adrenal function

Active oxygens produced by low-dose irradiation can induce the synthesis of proteins involved in the active oxygen reduction path and can activate the chemical self-defense mechanisms in vivo, which can alleviate the injuries caused by active oxygens themselves. The following findings suggests that it is not only by low-dose irradiation that can induce the responses described above but also by any physical and/or chemical stresses which can produce small amount of active oxygens in vivo. 1. X-irradiation with 0.5 Gy and/or water immersion and restraint induced heat shock protein (HSP)70 in rat stomach cells. HSP70 was also induced in adrenal cells by paraquat administration. 2. The SOD activity in pancreas of rat significantly increased by #gamma#-irradiation with 0.5 Gy. 0.5 Gy irradiation preceeding the alloxan administration significantly suppressed the increase in pancreatic lipid peroxides and in blood glucose levels, degranulation in #beta# cells, and ...

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121

Pituitary function following megavoltage therapy for Cushings' disease; long term follow up

A simple, short and sensitive direct radio-immunoassay technique for the determination of salivary cortisol concentration was employed to assess saliva as a medium for evaluating cortisol response during endocrine testing in 9 controls and 40 patients. Results in controls suggested that an adequate salivary cortisol response to insulin hypoglycaemia was an increase of 150% above the basal value with a minimum peak of 15 nmol/l. Thirty-three patients were classified as being either good or poor responders to insulin hypoglycaemia on the basis of criteria for plasma cortisol levels. When the defined salivary cortisol response was used for assessment, all 33 patients were correctly categorized into the same response groups. The salivary cortisol response to intramuscular tetracosactin in 3 patients and an intravenous dexamethasone infusion in 4 patients confirmed the value of saliva as an assay medium. These studies show that the salivary cortisol response parallels that of total plasma ...

1984-05-01

122

Pituitary function following megavoltage therapy for Cushings' disease; long term follow up

Eight patients who had received megavoltage therapy for Cushings' disease 5-12 years previously have been reviewed. The long term response to this therapy was assessed with respect to efficacy of treatment in inducing continued remission and disturbance of hypothalamic-pituitary function. One patient showed clear evidence of relapse of Cushings' disease. One patient had unequivocal hypopituitarism. Basal levels of growth hormone (GH), TSH, LH, and FSH were not statistically different from controls, but provocative testing revealed significant abnormalities of response of cortisol/ACTH, GH, prolactin and LH. Six out of eight patients had absent diurnal cortisol variation and five patients had elevated serum prolactin levels. Thus, in this group of patients normal pituitary-adrenal function has not been satisfactorily restored. It is clear that significant disturbances of hypothalamic-pituitary function follow megavoltage therapy and these may progress to overt ...

1985-01-01

123

Mapping of the gene encoding the melanocortin-1 ([alpha]-melanocyte stimulating hormone) receptor (MC1R) to human chromosome 16q24. 3 by fluorescence in situ hybridization

1985-02-01

124

Ganglioneuromas in childhood: MRI and CT characteristics

[alpha]-Melanocyte stimulating hormone ([alpha]-MSH), a hormone originally named for its ability to regulate pigmentation of melanocytes, is a 13-amino-acid post-translational product of the pro-opiomelanocortin (POMC) gene. [alpha]-MSH and the other products of POMC processing, which share the core heptapeptide amino acid sequence Met-Glu (Gly)-His-Phe-Arg-Trp-Gly (Asp), the adrenocorticotropic hormone (ACTH), [beta]-MSH, and [gamma]-MSH, are collectively referred to as melanocortins. While best known for their effects on the melanocyte (pigmentation) and adrenal cortical cells (steroidogenesis), melanocortins have been postulated to function in diverse activities, including enhancement of learning and memory, control of the cardiovascular system, analgesia, thermoregulation, immunomodulation, parturition, and neurotrophism. To identify the chromosomal band encoding the human melanocortin-1 receptor gene, 1 [mu]g of an EMBL clone coding region of the human MC1R ...

1994-01-15

125

Atrial natriuretic peptide receptor heterogeneity and effects on cyclic GMP accumulation

Purpose: The aim of this study was to demonstrate the typical appearance of ganglioneuromas in computer-assisted tomography (CT), and magnetic resonance imaging (MRI). Material and methods: Retrospective analysis of diagnostic imaging (9 CT, 6 MRI) in 9 children aged 3 to 15 years with the histological diagnosis of ganglioneuroma. Results: The tomographies showed large (max. 13.4 cm in diameter) round or oval tumors with sharp delineation. The sites of the tumors were the retroperitoneum (5), the mediastinum (3), and the adrenal gland (1). Intraspinal tumor involvement occurred in 4 cases. On comparing CT with MRI, MRI was more accurate in defining the intraspinal involvement. The ganglioneuromas were of hypodense appearance in the native CT scan and showed moderate enhancement upon administration of contrast media. In five patients tumor calcifications with a disseminated sprinkled pattern were seen in CT. In MRI T_1-weighted scans the tumors were homogeneous and ...

2000-05-01

126

Spatial distribution of disease: three case studies

The effects of atrial natriuretic peptide (ANP), oxytocin (OT) and vasopressin (AVP) on guanylate cyclase activity and cyclic GMP accumulation were examined, since these hormones appear to be intimately associated with blood pressure and intravascular volume homeostasis. ANP was found to increase cyclic GMP accumulation in ten cell culture systems, which were derived from blood vessels, adrenal cortex, kidney, lung, testes and mammary gland. ANP receptors were characterized in intact cultured cells using {sup 125}I-ANP{sub 8-33}. Specific {sup 125}I-ANP binding was saturable and of high affinity. Scratchard analysis of the binding data for all cell types exhibited a straight line, indicating that these cells possessed a single class of binding sites. Despite the presence of linear Scatchard plots, these studies demonstrated that cultured cells possess two functionally and physically distinct ANP-binding sites. Most of the ANP-binding sites in cultured cells have a ...

1988-01-01

127

One-year follow-up of the effects of sildenafil on pulmonary arterial hypertension and veno-occlusive disease

Maps transformed so as to have constant density of residential population were used to analyze the spatial distribution of disease in three specific areas. Each area had received recent attention because of suspected environmental pollution. The area adjacent to the Rocky Flats Facility (CO) was examined to identify any association between possible plutonium releases and increases in lung cancer or leukemia incidence. The industrial area of northern Contra Costa County (CA) was studied to explore a relationship between petrochemical industrial emissions and histologic-specific lung cancers. Finally, a suspected increase in the risk of congenital cardiac defects possibly related to pollution of the Santa Clara County (CA) water supply was investigated. No evidence of elevated risk of disease was found to be associated with either the Rocky Flats Facility or the polluted water of Santa Clara County. An increase in lung cancer, found by other investigators in earlier ...

128

Scientific Electronic Library Online (English)

Abstract in english We hypothesized that chronic oral administration of the phosphodiesterase-5 inhibitor sildenafil could improve the exercise capacity and pulmonary hemodynamics in patients with pulmonary arterial hypertension (PAH) on the basis of previous short-term studies. We tested this hypothesis in 14 subjects with PAH, including seven patients with the idiopathic form and seven patients with atrial septal defects, but no other congenital heart abnormalities. Patients were subjected (more) to a 6-min walk test and dyspnea was graded according to the Borg scale. Pulmonary flow and pressures were measured by Doppler echocardiography. Patients were given sildenafil, 75 mg orally three times a day, and followed up for 1 year. Sildenafil therapy resulted in the following changes: increase in the 6-min walk distance from a median value of 387 m (range 0 to 484 m) to 462 m (range 408 to 588 m; P

2005-02-01

129

Identification, characterization, and chromosomal localization of the human homolog (hES) of ES/130

Computed tomography of the orbit

The chicken extracellular matrix glycoprotein ES/130 is necessary for epithelial-mesenchymal transformation in the developing hear and is also expressed in noncardiac chicken tissues such as limb and notochord. We have identified hES, the human homology of chicken ES/130. Fluorescence in situ hybridization analysis (FISH) localizes hES to human chromosome 20p11.2-p12. FISH analyses of individuals with 20p12 deletions and affected by Alagille syndrome exclude hES as a candidate gene for this disorder. Reverse transcriptase-polymerase chain reaction studies reveal that hES is expressed in both fetal and adult human tissues and that hES expression in the left ventricle is increased in the failing adult heart. Further studies will evaluate how hES mutations may relate to congenital human cardiac and skeletal anomalies as well as cardiac remodeling in the adult. 16 refs., 2 figs.

1996-08-01

130

Assisted reproductive technologies for male factor infertility.

In less than a decade computed tomography (CT scanning) had a profound impact on diagnostic radiology. Radiology of the orbit is no exception. As early as 1973, reports published in the radiological literature indicated that this new noninvasive imaging method was a highly effective way of demonstrating intraorbital mass lesions. As CT scanners became widely available, computed tomography became a significant adjunct to ophthalmological diagnosis. Today the main indications for CT scanning of the orbit are: (1) suspected mass lesions, most frequently presenting as exophthalmos, (2) orbital trauma, including foreign bodies, (3) some congenital anomalies, and (4) suspicion of extension into the orbit of extraorbital disease processes. Along with ultrasonography, another new noninvasive imaging technique, CT has replaced a number of more invasive and often less effective diagnostic methods, such as orbital pneumography, venography, and arteriography as major imaging ...

1981-01-01

131

Health impact assessment of waste management facilities in three European countries

Experience in the diagnosis and treatment of infertility has led to an increased understanding of the prominent role male factors play in many couples' inability to conceive. While many forms of male factor infertility are amenable to treatment, for some patients there is no corrective therapy available. For this reason, a great deal of attention has been focused on developing technologies for semen processing and sperm utilization in cases of decreased semen quality. One area of research is the development of more sophisticated methods of assessing sperm function, as well as methods of sperm preparation. Progress is also being made in the application of technologies such as intrauterine insemination and in vitro fertilization in the treatment of male factor infertility. Such developments have improved prospects for fertility among patients suffering from decreased semen quality, as well as those suffering from such disorders as ejaculatory failure and congenital ...

1991-10-01

132

Ganglioneuromas in childhood: MRI and CT characteristics; Ganglioneurome im Kindesalter: CT- und MRT-Charakteristika

BackgroundPolicies on waste disposal in Europe are heterogeneous and rapidly changing, with potential health implications that are largely unknown. We conducted a health impact assessment of landfilling and incineration in three European countries: Italy, Slovakia and England.MethodsA total of 49 (Italy), 2 (Slovakia), and 11 (England) incinerators were operating in 2001 while for landfills the figures were 619, 121 and 232, respectively. The study population consisted of residents living within 3 km of an incinerator and 2 km of a landfill. Excess risk estimates from epidemiological studies were used, combined with air pollution dispersion modelling for particulate matter (PM10) and nitrogen dioxide (NO2). For incinerators, we estimated attributable cancer incidence and years of life lost (YoLL), while for landfills we estimated attributable cases of congenital anomalies and low birth weight infants.ResultsAbout 1,000,000, 16,000, and 1,200,000 subjects lived ...

2011-06-02

133

Evaluation of left ventricular volumes in patients with congenital heart disease and abnormal left ventricular geometry. Comparison of MRI and transthoraic 3-dimensional echocardiography; Vergleich der transthorakalen 3D-Echokardiographie mit der MRT zur Bestimmung linksventrikulaerer Volumina bei Patienten mit pathologischer Ventrikelgeometrie aufgrund angeborener Herzfehler

Purpose: The aim of this study was to demonstrate the typical appearance of ganglioneuromas in computer-assisted tomography (CT), and magnetic resonance imaging (MRI). Material and methods: Retrospective analysis of diagnostic imaging (9 CT, 6 MRI) in 9 children aged 3 to 15 years with the histological diagnosis of ganglioneuroma. Results: The tomographies showed large (max. 13.4 cm in diameter) round or oval tumors with sharp delineation. The sites of the tumors were the retroperitoneum (5), the mediastinum (3), and the adrenal gland (1). Intraspinal tumor involvement occurred in 4 cases. On comparing CT with MRI, MRI was more accurate in defining the intraspinal involvement. The ganglioneuromas were of hypodense appearance in the native CT scan and showed moderate enhancement upon administration of contrast media. In five patients tumor calcifications with a disseminated sprinkled pattern were seen in CT. In MRI T{sub 1}-weighted scans the tumors were homogeneous ...

2000-05-01

134

Pituitary gland height evaluated by MR in patients with {beta}-thalassemia major: a marker of pituitary gland function

Purpose: To assess the new method of 3-dimensional echocardiography in comparison to the 'gold standard' MRI as to its ability to calculate left ventricular volumes in patients with congenital heart disease. Materials and methods: Eighteen patients between the ages of 3.9 to 37.3 years (mean: 12.8{+-}9.7) with a geometrically abnormal left ventricle were examined using a 1.5 T scanner with a fast gradient-echo sequence (TR=14 ms, TE=2.6-2.9 ms, FOV=300-400 mm, flip angle=20 , matrix=128:256, slice thickness=5 mm, retrospective gating) in multislice-multiphase technique. Transthoracic 3D-echocardiography was performed with a 3.5 MHz transducer and a Tomtec {sup trademark} (Munich, Germany) system for 3D reconstruction. Results: Volume calculation was possible in all patients with 3D-echocardiography, but the muscle mass calculation only succeeded in 11 to 18 patients (61%) due to inadequate visualization of the entire myocardium. Comparing MRI and ...

2003-07-01

135

Inhibitor of DNA synthesis is present in normal chicken serum

In transfusion-dependent {beta}-thalassemia major, increased iron deposition in the pituitary gland has a cytotoxic effect leading mainly to hypogonadotropic hypogonadism. Our purpose was to assess in these patients the height of the pituitary gland and to evaluate whether it represents a marker of pituitary gland function. In 29 patients with {beta}-thalassemia major and 35 age- and gender-matched controls the pituitary gland height was evaluated in a midline sagittal scan using a spin echo T1-weighted (500/20 TR/TE) sequence. In all patients, an extensive endocrine evaluation was performed, including measurements of spontaneous and stimulated levels of gonadotropins, thyroid hormones, growth hormone, insulin-like growth factor, and adrenal hormones. The pituitary gland height was lower in thalassemic patients with hypogonadotropic hypogonadism (n=15) (mean 3.48; SD 0.46) than in the age- and gender-matched controls (mean 6.29; SD 0.77), (P<0.001). No ...

2001-12-01

136

Impaired neonatal survival of pro-opiomelanocortin null mutants.

The authors have found that heat-inactivated serum (57/sup 0/C for 1 hour) from normal chickens reduces the proliferation of mitogen-stimulated chicken and murine splenocytes as well as some transformed mammalian lymphoblastoid cell lines. Greater than a 50% reduction in /sup 3/H-thymidine incorporation was observed when concanavalin A (Con A)-activated chicken splenocytes that were cultured in the presence of 10% autologous or heterologous serum were compared to mitogen-stimulated cells cultured in the absence of serum. Normal chicken serum (10%) also caused greater than 95% suppression of /sup 3/H-thymidine incorporation by bovine (EBL-1 and BL-3) and gibbon ape (MLA 144) transformed lymphoblastoid cell lines. The only cell line tested that was not inhibited by chicken serum was an IL-2-dependent, murine cell line. Chicken serum also inhibited both /sup 3/H-thymidine incorporation and IL-2 synthesis by Con A-activated murine splenocytes. Suppression was caused by actions other than ...

1986-03-05

137

Medical treatment of nystagmus and ocular motor disorders.

Intercrosses of heterozygous pro-opiomelanocortin (POMC) mice result in homozygous null progeny at lower frequencies than expected. Genotyping offspring at pre-, peri-, and postnatal stages revealed that over half of homozygous null mutants die in the early postnatal stages. To investigate the reasons for this early postnatal lethality, we analyzed in detail different parameters in the initial hours after birth. POMC null mutants born to heterozygous dams presented at birth with corticosterone levels no different from wildtype littermates, were euglycemic, and had normal liver glycogen stores. However, already 30 min after birth corticosterone levels dropped by 80% and were undetectable thereafter, while corticosterone levels in wildtype animals increased during postnatal hours. Circulating adrenaline was almost below detection 1h after birth. Blood glucose levels fell sharply in all genotypes within 30 min after birth; however, wildtype and heterozygous pups overcame hypoglycemia ...

2010-12-22

138

MRI in the diagnosis of non-compacted ventricular Myocardium (NCVM) compared to echocardiography

An increased compendium of drugs useful in ocular motor system dysfunction has expanded our capacity to treat selected ocular motility disorders. Adjunctive therapeutic modes (e.g., Fresnel prisms and orthoptic exercises) can also be beneficial. PAN and see-saw nystagmus can be treated with baclofen. Downbeat nystagmus may respond to clonazepam therapy, and prisms may help if the nystagmus can be modified with convergence. Congenital nystagmus may respond minimally to drugs (e.g., baclofen), but prisms or surgical procedures, or both, are still the primary treatment modalities. Innovar may be helpful in patients with severe, incapacitating vestibular disorders, and scopolamine alone or in combination with promethazine may be beneficial in patients with milder ambulatory acute peripheral vestibular disorders. Benign positional vertigo is best treated initially with positional exercises before drug therapy is instituted. Opsoclonus and ocular flutter have been ...

1986-01-01

139

Long-Term Follow-Up of Percutaneous Balloon Angioplasty in Adult Aortic Coarctation

Purpose: To report the detection of con-compacted ventricular myocardium (NVCM) with MRI compared to echocardiography in 8 patients. Material and methods: Non-compaction of the ventricular myocardium is a congenital disorder characterized by an altered structure of the myocardial wall resulting from an intrauterine arrest in endomyocardial embryogenesis. The morphological findings consist of a prominent meshwork of multiple myocardial trabeculations and deep intertrabecular recesses, communicating with the left ventricular cavity. 8 consecutive patients (mean age 7.3 years) with clinical and echocardiographic signs of NCVM were examined by MRI (1.5 T, Vision, Siemens) in short axis and 2- and 4-chamber views, using T_1-weighted TSE and Cine-GRE in 6 patients and true FISP sequences in 2 patients. MRI and echocardiography were evaluated for visibility, signs of NCVM and involvement of myocardial wall segments. Thickness was measured for non-compacted and compacted ...

2003-09-01

140

Four years of North American registry home parenteral nutrition outcome data and their implications for patient management

Purpose: To assess long-term outcomes following percutaneous transluminal angioplasty (PTA) of congenital aortic coarctation in adults.Methods: Seventeen patients underwent PTA for symptomatic adult coarctation of the aorta. Sixteen patients, with a mean age of 28 years (range 15-60 years), were reviewed at a mean interval after angioplasty of 7.3 years (range 1.5-11 years). Assessment included magnetic resonance imaging (MRI), Doppler echocardiography, and clinical examination. Current clinical measurements were compared with pre- and immediate post-angioplasty measurements.Results: At follow-up 16 patients were alive and well. The patient not included in follow-up had undergone surgical repair and excision of the coarctation segment following PTA. Mean brachial systolic blood pressure for the group decreased from 174 mmHg before angioplasty to 130 mmHg at follow-up (p 0.0001). The mean gradient had fallen significantly from 50.9 to 17.8 at follow-up (p = 0.001). ...

2000-09-01

1 2 3 4 5 6 7 8

141

Corrected transposition of the great arteries

The OASIS Registry started annual collection of longitudinal data on patients on home parenteral nutrition (HPN) in 1984. This report describes outcome profiles on 1594 HPN patients in seven disease categories. Analysis showed clinical outcome was principally a reflection of the underlying diagnosis. Patients with Crohn's disease, ischemic bowel disease, motility disorders, radiation enteritis, and congenital bowel dysfunction all had a fairly long-term clinical outcome, whereas those with active cancer and acquired immunodeficiency syndrome (AIDS) had a short-term outcome. The long-term group had a 3-year survival rate of 65 to 80%, they averaged 2.6 complications requiring hospitalization per year, and 49% experienced complete rehabilitation. The short-term group had a mean survival of 6 months; they averaged 4.6 complications per year and about 15% experienced complete rehabilitation. The registry data also indicated HPN was used for 19,700 patients in 1987 with ...

142

Case report: Denys- Drash syndrome.

The corrected transposition of the great arteries is an usual congenital cardiac malformation, which consists of transposition of great arteries and ventricular inversion, and which is caused by abnormal development of conotruncus and ventricular looping. High frequency of associated cardiac malformations makes it difficult to get accurate morphologic diagnosis. A total of 18 cases of corrected transposition of the great arteries is presented, in which cardiac catheterization and angiocardiography were done at the Department of Radiology, Seoul National University Hospital between September 1976 and June 1981. The clinical, radiographic, and operative findings with the emphasis on the angiocardiographic findings were analyzed. The results are as follows: 1. Among 18 cases, 13 cases have normal cardiac position, 2 cases have dextrocardia with situs solitus, 2 cases have dextrocardia with situs inversus and 1 case has levocardia with situs inversus. 2. Segmental sets ...

1981-12-01

143

ATRX ADD domain links an atypical histone methylation recognition mechanism to human mental-retardation syndrome

BACKGROUND: Denys-Drash Syndrome (DDS) is an uncommon disorder that appears sporadically and in rare cases may be inherited as an autosomal dominant trait It manifests either at birth or within the first year of life and typically consists of the triad of congenital nephropathy, Wilms tumour and intersex disorder. CASE REPORT: A 10 year-old Caucasian girl was referred to the Dental Department, at Glasgow Royal Hospital for Sick Children by her Paediatric Nephrologist Consultant. The patient was being teased by her peers over her markedly discoloured teeth. The dental history revealed that the patient was a regular dental attendee from an early age. She was dentally anxious having only experienced dental treatment under general anaesthesia (GA) when she was 4 years old. Apparently her primary dentition also showed a generalised discolouration. TREATMENT: This consisted of multiple visits for diet analysis and tooth brushing instruction with the use of disclosing ...

2007-12-01

144

A radiologic analysis and comparative evaluation of hysterosalpingographic and laparoscopic findings in infertile patients

ATR-X (alpha-thalassemia/mental retardation, X-linked) syndrome is a human congenital disorder that causes severe intellectual disabilities. Mutations in the ATRX gene, which encodes an ATP-dependent chromatin-remodeler, are responsible for the syndrome. Approximately 50% of the missense mutations in affected persons are clustered in a cysteine-rich domain termed ADD (ATRX-DNMT3-DNMT3L, ADD{sub ATRX}), whose function has remained elusive. Here we identify ADD{sub ATRX} as a previously unknown histone H3-binding module, whose binding is promoted by lysine 9 trimethylation (H3K9me3) but inhibited by lysine 4 trimethylation (H3K4me3). The cocrystal structure of ADD{sub ATRX} bound to H3{sub 1-15}K9me3 peptide reveals an atypical composite H3K9me3-binding pocket, which is distinct from the conventional trimethyllysine-binding aromatic cage. Notably, H3K9me3-pocket mutants and ATR-X syndrome mutants are defective in both H3K9me3 binding and localization at ...

2011-07-19

145

Sex and seasonal differences in the rate of cell proliferation in the dentate gyrus of adult wild meadow voles.

Hystreosalpingogram of 375 patients with infertility were reviewed with reference to radiological feature of each pathology and a comparative study of hysterosalpingography and laparoscopy in 36 patients was presented. This results were as follows;1. In 375 cases of infertility, 191 cases (50.9%) were primary infertility and 181 cases (49.1%) were secondary infertility. 2. The peak age distribution was 26 to 30 year old group in 211 cases (56.3%), and the peak duration of infertility was less than 3 years in 256 cases (68.3%). 3. Abnormal uterine findings were 115 cases (30.7%) and there were 73 cases of filling defects, 13 cases of irregularity, 12 cases of uterine flexion, 11 cases of congenital anomaly, and 9 cases of hypoplasia among them. 4. Abnormal tubal findings were 196 cases (52.3%) and there were 103 cases of simple obstruction, 69 cases of hydrosalpinx, and 50 cases of peritubal adhesion among them. 5. Intravasation of contrast media was noted in 42 ...

1984-09-15

146

New procedures. Comprehensive staging of lung cancer by MRI; Neue Verfahren. Umfassendes Staging des Lungenkarzinoms mit der MRT

In order to study the neurobiological basis of seasonal changes in hippocampal structure and function, the rate of cell proliferation was examined in male and female wild meadow voles captured during different seasons. We found that the number of [3H]thymidine-labeled cells varied across the seasons and across sex in the meadow vole. Non-breeding female meadow voles had a higher rate of cell proliferation and cell death than males captured during either season or breeding females. These seasonal changes in the female meadow vole were associated with both fluctuating levels of adrenal steroids and gonadal steroids. Estradiol level was highly correlated with both the number of [3H]thymidine-labeled cells and the number of pyknotic cells in female meadow voles, with high levels of estradiol being associated with low levels of cell proliferation and cell death. Corticosterone level was associated with the number of [3H]thymidine-labeled cells in the hilus of female ...

1999-03-01

147

MRI in the diagnosis of non-compacted ventricular Myocardium (NCVM) compared to echocardiography; MR-Tomographie in der morphologischen Darstellung der isolierten non-compaction des linksventrikulaeren Myokards (NCVM)

2010-08-15

148