MedWorm: Addison's Disease

Adrenal function in newborns undergoing surgery

Journal of Perinatology — Thu, 18 Mar 2010 00:00:00 +0100

Authors: E F Fernandez, R Montman & K L Watterberg (Source: Journal of Perinatology)

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Intra familial phenotypical variations in adrenoleukodystrophy

Neurology India — Tue, 09 Mar 2010 15:01:56 +0100

We report a family of three siblings diagnosed with ALD confirmed with the mutations in ABCD1 gene having phenotypical variability ranging from pure adrenal insufficiency to progressive neurodegeneration in the same family. The mother was identified as the carrier and maternal uncle was diagnosed with Adrenomyeloneuropathy. We discuss the variable presentation in our family and the possible causes of phenotypical variability. (Source: Neurology India)

Two Patients with an Identical Novel Mutation in the AAAS Gene and Similar Phenotype of Triple A (Allgrove) Syndrome

Experimental and Clinical Endocrinology and Diabetes — Thu, 04 Mar 2010 14:23:38 +0100

Exp Clin Endocrinol DiabetesDOI: 10.1055/s-0030-1247516Abstract Triple A syndrome, also known as Allgrove syndrome, is a rare autosomal recessive disorder characterized by three cardinal symptoms: adrenal insufficiency due to ACTH insensitivity, achalasia and alacrima. Various progressive neurological abnormalities and skin changes have been described in association with the syndrome. The disease is caused by mutation in the gene on chromosome 12q13. encodes a protein named ALADIN which is part of the nuclear pore complex (NPC). The mislocalization of mutated ALADIN proteins in the cytoplasm and/or the nucleus results in an impaired protein function. Phenotypes of previously reported patients with triple A syndrome varied within and between affected families so that no genotype-phenotype c...

National UK audit of the Short Synacthen(R) Test

Annals of Clinical Biochemistry — Tue, 02 Mar 2010 17:36:02 +0100

Conclusions The audit has shown that though the preanalytical procedures were similar in most laboratories, there is a requirement to recognize the effect that method bias may have on the reference ranges and consequently on the diagnosis of adrenal insufficiency. There is a need to develop consensus guidelines, which can aid both clinicians and laboratories. (Source: Annals of Clinical Biochemistry)

Antithymocyte globulin: Cytomegalovirus infection leading to adrenal insufficiency?: case report

Reactions — Mon, 01 Mar 2010 14:50:10 +0100

(Source: Reactions)

Cushing’s syndrome and adrenal insufficiency after intradermal triamcinolone acetonide for keloid scars

European Journal of Pediatrics — Thu, 25 Feb 2010 20:31:47 +0100

Abstract A 6-year-old girl was admitted to our hospital for excessive weight gain. On presentation, she had a typical Cushingoid appearance and hypertension. Laboratory analysis was indicative for an exogenous glucocorticoid source, showing values that were low for serum cortisol and adrenocorticotropin and for 24-h urinary glucocorticoid (metabolite) excretion. Five and 2 months before presentation, she was treated with intradermal triamcinolone acetonide for keloid scars. Recovery of intrinsic cortisol production occurred 12 months after the last steroid dose. Intradermal triamcinolone acetonide for keloid scars in children should be used with care taking into account the here reported complication. Content Type Journal ArticleCategory Short ReportDOI 10.10...

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An unusual case of hyponatraemia

Nephrology Dialysis Transplantation — Thu, 25 Feb 2010 08:19:39 +0100

The differential diagnosis of hyponatraemia is manifold and includes hormonal disorders such as primary adrenal insufficiency or hypothyroidism. The diagnosis of adrenal insufficiency is always suggestive in cases of hypotension associated with hyponatraemia, hyperkalaemia and metabolic acidosis. We herein report a case of severe hyponatraemia in a patient with Addison's disease. The underlying cause was disseminated adrenal tuberculosis without any evidence of other organ involvement. To date, tuberculosis remains a frequent cause of adrenal insufficiency although the pathophysiology of adrenal tropism is poorly understood. (Source: Nephrology Dialysis Transplantation)

Quartan malaria-associated childhood nephrotic syndrome: now a rare clinical entity in malaria endemic Nigeria

Nephrology Dialysis Transplantation — Thu, 25 Feb 2010 08:19:38 +0100

Conclusion. Overall outcome of CNS has improved significantly compared to the 1960s and 1970s when the poor outcome of QMN was the predominant glomerular lesion in Nigeria. While quartan malaria-associated nephrotic syndrome has become a rare clinical entity, SLE, SCA and HBV infection have become the major secondary aetiologies of CNS in Nigeria. (Source: Nephrology Dialysis Transplantation)

Closed Zone III Rupture of the Flexor Digitorum Profundus Tendons of the Right Index, Long, and Ring Fingers in a Bowler: Gutterball Syndrome

Hand — Wed, 24 Feb 2010 06:55:15 +0100

Abstract Zone III flexor tendon injuries are relatively rare in comparison to other flexor tendon injuries in zones I, II, IV, and V. Often, these are open injuries resulting from an electrical device like a saw; however, closed injures are even rarer, and those mid-substance ruptures resulting from bowling with no evidence of underlying tendinopathy from diseases like gout are highly unusual. The principles underlying tendon repair remain the same regardless of the etiology. In this case, we delineate some of the options and stress the guiding principles of the various methods available in this interesting and unusual case. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s11552-010-9259-7Authors Srdjan Andrei Ostric, Midwest PRS 5645 W. Addison St. S...

Cortisone replacement therapy in endocrine disorders – quality of self-care

Journal of Evaluation in Clinical Practice — Mon, 22 Feb 2010 00:00:00 +0100

Conclusion Information transfer by doctors is the main source of information for the patients. The low patient numbers make the development of structured education programmes unlikely. Given that only the half of the answers in the 'knowledge' section of the questionnaire were correct, the available media could contribute to the improvement of information transfer. (Source: Journal of Evaluation in Clinical Practice)

HIMSS10 NewsWire: MEDHOST launches OpCenter, addresses inpatient throughput

Healthcare IT News Press Releases — Thu, 18 Feb 2010 21:16:25 +0100

MEDHOST, provider of EDIS and healthcare throughput solutions, today announced the launch of OpCenter, its new executive decision support software solutions. OpCenter addresses inpatient flow and bed management issues by providing executives with reliable, real-time information so they can make informed decisions about how to dramatically improve their hospital’s operations and increase patient satisfaction. (Source: Healthcare IT News Press Releases)

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Critical role of natural killer cells in the rejection of human hepatocytes after xenotransplantation into immunodeficient mice

Transplant International — Wed, 17 Feb 2010 00:00:00 +0100

The severe combined immunodeficiency/albumin linked-urokinase type plasminogen activator (SCID/Alb-uPA) human liver chimeric mouse model has added a new dimension to studies of liver based human diseases and has important potential for study of human hepatic drug metabolism. However, it remains unclear if natural killer (NK) cell in SCID/Alb-uPA mice has an important negative impact on engraftment and expansion of human hepatocytes after transplantation. Here, we explore the role of mouse NK cells in the rejection of transplanted human hepatocytes in SCID/Alb-uPA mice. We assessed NK cell activity in vivo, using 125I-iodo-2'-deoxyuridine incorporation assay. Low serum human alpha-1 antitrypsin (hAAT, 100 [mu]g/ml) recipients accepted MHC class I knockout marrow, indicating a correlation be...

Flexion contractures possibly reflect the existence of hypocortisolism: two case reports

Journal of Neurology — Mon, 15 Feb 2010 17:59:36 +0100

Abstract The diagnosis of adrenal insufficiency is discussed in case of low blood pressure and digestive symptoms. Rare inaugural abdomino-crural muscle contracture can be a misleading symptom. Here we report two new cases. A 50-year-old woman presenting a leaning forward walking attitude and negligence for the past 2 months was referred to the neurologic unit. Abdomino-crural contracture, clinical hypogonadism, and hyponatremia directed towards a panhypopituitarism, which was confirmed by subsequent investigations. Pituitary MRI found an empty sella turcica. The outcome was dramatic after hormone replacement therapy, with drawing up of the trunk and re-establishment of walking after a few days. The second case is a 58-year-old man, hospitalized with altered general c...

Fentanyl: Secondary adrenal insufficiency: case report

Reactions — Mon, 15 Feb 2010 14:06:03 +0100

(Source: Reactions)

Cushing Syndrome in the McCune-Albright Syndrome.

The Journal of Clinical Endocrinology and Metabolism — Mon, 15 Feb 2010 00:00:00 +0100

Conclusions: Comorbid heart and liver disease were poor prognostic markers and may indicate the need for prompt adrenalectomy. The high incidence of cognitive disorders indicates a need for close developmental follow-up and parental counseling. Patients with spontaneous resolution of CS may develop adrenal insufficiency, and they require long-term monitoring. PMID: 20157193 [PubMed - as supplied by publisher] (Source: The Journal of Clinical Endocrinology and Metabolism)

Halonitromethanes formation in wastewater treatment plant effluents.

Chemosphere — Thu, 11 Feb 2010 00:00:00 +0100

This study investigated the formation and occurrence of HNMs under different disinfection scenarios and the presence of their precursors in municipal wastewater treatment plant (WWTPs) effluents. Formation potential tests performed on WWTP effluents revealed that HNM formation occurred in the order of ozonation-chlorination>>ozonation-chloramination>chlorination>chloramination. Ozonation alone did not produce any HNM. Municipal WWTP effluents contained some reactive HNM precursors, possibly the by-products of biological treatment processes and/or some moiety of industry or household origin. No effects of nitrate on the formation of HNMs were observed in this study, and nitrification in WWTPs appears to remove appreciable portion of HNM precursors, especially those reactive to c...

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HPA-suppressive effects of aqueous clobetasol propionate in the treatment of patients with oral lichen planus

Journal of the European Academy of Dermatology and Venereology — Wed, 10 Feb 2010 00:00:00 +0100

Conclusions Hypothalamus[ndash]pituitary[ndash]adrenal inhibition is substantial during initial treatment with aqueous CP three times daily. (Source: Journal of the European Academy of Dermatology and Venereology)

A coding variant in NLRP1is associated with autoimmune Addison;s disease.

Human Immunology — Tue, 09 Feb 2010 00:00:00 +0100

In conclusion, this study confirms an association between the coding polymorphism in NLRP1 and AAD. PMID: 20152874 [PubMed - as supplied by publisher] (Source: Human Immunology)

Determining Relevant Cortisol Concentrations in Critically Ill Patients

Critical Care — Mon, 08 Feb 2010 00:00:00 +0100

: The importance of adrenal function to survival in critically ill patients has been established; however, identifying the best method to diagnose adrenal insufficiency has been problematic. Multiple methods of determining adrenal function have been developed, each with its advantages and disadvantages. Serum free cortisol levels are probably the most accurate though obtaining this result is technically demanding. Cohen et al (1) investigated the feasibility of measuring tissue cortisol levels in burn patients, and investigated if tissue cortisol levels could be used as a surrogate for plasma free cortisol levels. (Source: Critical Care)

Inositol hexakisphosphate inhibits mineralization of MC3T3-E1 osteoblast cultures

Bone — Mon, 01 Feb 2010 00:00:00 +0100

In this study, we used MC3T3-E1 osteoblast cultures to examine the effects of exogenous IP6 on osteoblast function and matrix mineralization. IP6 at physiologic concentrations caused a dose-dependent inhibition of mineralization without affecting cell viability, proliferation or collagen deposition. Osteoblast differentiation markers, including tissue-nonspecific alkaline phosphatase activity, bone sialoprotein and osteocalcin mRNA levels, were not adversely affected by IP6 treatment. On the other hand, IP6 markedly increased protein and mRNA levels of osteopontin, a potent inhibitor of crystal growth and matrix mineralization. Inositol alone (without phosphate), as well as inositol hexakis-sulphate, a compound with a high negative charge similar to IP6, had no effect on mineralization or ...

Postnatal Changes in Adrenal Size in Very Low-Birth-Weight Infants: Sonographic Evaluation for the Prediction of Late-Onset Glucocorticoid-Responsive Circulatory Collapse

American Journal of Perinatology — Sat, 30 Jan 2010 02:51:22 +0100

Amer J PerinatolDOI: 10.1055/s-0030-1247604ABSTRACTWe investigated the postnatal pattern of changes in adrenal size in very low-birth-weight (VLBW) infants and its relation to late-onset glucocorticoid-responsive circulatory collapse (LGCC) that may be associated with adrenal insufficiency. In 36 VLBW infants born at <33 weeks' gestation, ultrasound examinations of postnatal changes in adrenal size during the first 3 weeks of life were performed. VLBW infants were classified into three groups: group A ( = 6), the actual adrenal area was greater than or equal to the predicted value at birth and unchanged at 3 weeks; group B ( = 24), the actual adrenal area was greater than or equal to the predicted value and decreased at 3 weeks; and group C ( = 6), th...

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Association of a dog leukocyte antigen class II haplotype with hypoadrenocorticism in Nova Scotia Duck Tolling Retrievers

Tissue Antigens — Thu, 28 Jan 2010 00:00:00 +0100

Canine hypoadrenocorticism (Addison's disease) is due to a deficiency of corticosteroids and mineralocorticoids produced by the adrenals. Although this is a relatively uncommon disease in the general dog population, some breeds, including the Nova Scotia Duck Tolling Retriever (NSDTR), are at increased risk for developing hypoadrenocorticism. A prior study has shown that the increased risk is due to a heritable component. This potentially lethal disorder is hypothesized to have an autoimmune etiology, thus the aim of this study was to determine whether genetic susceptibility to hypoadrenocorticism in NSDTRs is associated with genes of the canine major histocompatibility complex [MHC; dog leukocyte antigen system (DLA)]. Samples were collected from NSDTRs diagnosed with hypoadrenocorticism ...

Incidence of Biochemical and Clinical Adrenal Insufficiency in Trauma Patients

Journal of Surgical Research — Mon, 25 Jan 2010 16:42:35 +0100

Background: Acute adrenal insufficiency (AI) is a condition frequently expressed by critically ill patients. This is a mayor health concern, resulting in severe morbidity and increased mortality if prompt diagnosis is not made. Underlying risk factors that led to acute AI include, but not limited to: age, co-morbidities, mechanism of trauma, EBL, TISS, surgeries, etc. The reported incidence of AI varies widely (0-77%) depending on the population of patients studied and the diagnostic criteria, but the incidence in trauma patients presenting AI has yet to be determined. Purpose: To determine the incidence of acute AI in trauma patients; find out the morbidity rates and associated factors and evaluate the possible correlation between biochemical predictors (laboratory data) and the occurrenc...

Diabetes Mellitus: Observations, Theoretical and Practical: Bessman SP. J Pediatr 1960;56:191-203

The Journal of Pediatrics — Mon, 25 Jan 2010 15:34:19 +0100

In this article, written by Bessman in 1959, emphasis is first placed on the limited number of diabetic children experiencing coma (ie, diabetic ketoacidosis) at diagnosis (2 of 20 patients, in the author's experience). Such small prevalence was due, according to the author, to the “detection programs….identifying most of the early cases.” Interestingly, in a recent study on diabetic children and adolescents, the prevalence of ketoacidosis was not negligible (25.5%). This prevalence, which has not significantly changed in the past 50 years, suggests the need for more effective measures to prevent diabetic ketoacidosis. A few years ago, a community intervention to raise awareness of the symptoms of diabetes was found to reduce the prevalence of diabetic ketoacidosis from 78% to 12.5%....

Prevalence and Relative Risk of Other Autoimmune Diseases in Subjects with Autoimmune Thyroid Disease

The American Journal of Medicine — Sun, 24 Jan 2010 14:28:53 +0100

Conclusion: This is one of the largest studies to date to quantify the risk of diagnosis of coexisting autoimmune diseases in more than 3000 index cases with well-characterized Graves' disease or Hashimoto's thyroiditis. These risks highlight the importance of screening for other autoimmune diagnoses if subjects with autoimmune thyroid disease present with new or nonspecific symptoms. (Source: The American Journal of Medicine)

Optimizing the diagnostic potential of blood pressure measurement in Addison's disease

QJM — Sat, 23 Jan 2010 09:15:21 +0100

(Source: QJM)

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Rifampicin: Addison's disease in an elderly patient: case report

Reactions — Tue, 19 Jan 2010 14:09:29 +0100

(Source: Reactions)

Iatrogenic adrenal insufficiency associated with calcipotriol–betamethasone topical combination in psoriasis

Clinical And Experimental Dermatology — Mon, 18 Jan 2010 00:00:00 +0100

(Source: Clinical And Experimental Dermatology)

Apoptosis in subicular neurons: A comparison between suicide and Addison's disease. - Printha K, Hulathduwa SR, Samarasinghe K, Suh YH, De Silva KR.

SafetyLit: All (Unduplicated) — Sun, 17 Jan 2010 15:27:03 +0100

Background: Stress and depression shows possible links to neuronal death in hippocampus. Subiculum plays a prominent role in limbic stress integration and direct effect of corticosteroids on subicular neurons needs to be defined to assess its subsequent im... (Source: SafetyLit: All (Unduplicated))

Congenital Lipoid Adrenal Hyperplasia: Functional Characterization of Three Novel Mutations in the STAR Gene.

The Journal of Clinical Endocrinology and Metabolism — Fri, 15 Jan 2010 00:00:00 +0100

Conclusion: The mutations p.N148K and p.P129fs cause adrenal insufficiency in both cases and lead to a disorder of sex development with complete sex reversal in the 46, XY case. The mutation p.Q128R, which is not relevant for the patient's phenotype, is the first reported variant showing a gain of function. We speculate that the substitution of hydrophilic glutamine with basic arginine at the surface of the molecule may accelerate cholesterol transfer. PMID: 20080861 [PubMed - as supplied by publisher] (Source: The Journal of Clinical Endocrinology and Metabolism)

The effects of far-infrared irradiation on the antioxidant activity of licorice (glycyrrhiza uralensis fisch) root

Journal of Food Biochemistry — Wed, 13 Jan 2010 00:00:00 +0100

Licorice (Glycyrrhiza uralensis Fisch) roots (LR) powders were far-infrared (FIR)-irradiated for 30 min at 120[sim]200C. After irradiation, a 50% ethanol extract of LR (LRE) (0.5 g/50 mL) was prepared, and total phenolic content (TPC), total flavanol content (TFC) and radical scavenging activity (RSA) were determined. The antioxidant activities of the LRE were significantly affected by FIR irradiation. For example, the FIR irradiation of LR at 120C increased the TPC, TFC and RSA of the LRE from 22.85 to 29.81 mg/g, 5.61 to 13.31 mg/g and 24.59 to 52.24%, respectively, as compared with the nonirradiated control. The main functional chemical components of LR such as glycyrrhizin, glycyrrhetic acid and liquiritin, were also significantly affected by irradiation. These results indicate that th...

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Management of Cushing's Syndrome due to Ectopic Adrenocorticotropin Secretion with 1,Ortho-1, Para'-Dichloro-Diphenyl-Dichloro-Ethane: Findings in 23 Patients from a Single Center.

The Journal of Clinical Endocrinology and Metabolism — Fri, 08 Jan 2010 00:00:00 +0100

Conclusion: With close monitoring, O,p'DDD could be a potent medical treatment for long-term control and management of EAS. PMID: 20061433 [PubMed - as supplied by publisher] (Source: The Journal of Clinical Endocrinology and Metabolism)

Adrenal Insufficiency in Newborns with Congenital Diaphragmatic Hernia

The Journal of Pediatrics — Fri, 08 Jan 2010 00:00:00 +0100

Newborns with congenital diaphragmatic hernia frequently have catecholamine-unresponsive systemic hypotension and respiratory failure. We found that adrenal insufficiency frequently complicates the clinical course of infants with congenital diaphragmatic hernia and was associated with increased severity of illness. (Source: The Journal of Pediatrics)

Triple A syndrome: A novel compound heterozygous mutation in the AAAS gene in an Italian patient without adrenal insufficiency

Journal of the Neurological Sciences — Wed, 06 Jan 2010 00:00:00 +0100

We describe a 42-year-old patient who presented with neuropathy and was found to have alacrima, achalasia, mild autonomic dysfunction with significant central and peripheral nervous system involvement. She was later diagnosed with oligosymptomatic triple A syndrome. Sequencing of the AAAS gene identified two heterozygous mutations within exon 14 and its donor splice site (p.L430F−c.1288C>T and c.1331+1G>T), one of which is novel. Allgrove syndrome should be suspected in patients with neurological impairment associated with two or more of the main symptoms (alacrima, achalasia or adrenal insufficiency). (Source: Journal of the Neurological Sciences)

Emotions and features of temperament in patients with Addison's disease.

Endokrynologia Polska — Fri, 01 Jan 2010 00:00:00 +0100

Conclusions: The temperamental characteristics and personal traits of patients with Addison's disease seem not to be useful in stressful events, and psychological support can be helpful in the effective therapy of these patients. (Pol J Endocrinol 2010; 61 (1): 90-92). PMID: 20205110 [PubMed - in process] (Source: Endokrynologia Polska)

[Isolation of pituitary autoantigen by affinity chromatography.]

Endokrynologia Polska — Thu, 31 Dec 2009 17:16:03 +0100

Conclusions: Isolation of pituitary autoantigen by affinity chromatography shown two different antigen proteins with 67 kDa and 55 kDa. PMID: 20041363 [PubMed - as supplied by publisher] (Source: Endokrynologia Polska)

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On the diagnosis and investigation of adrenal insufficiency in adults

Annals of Clinical Biochemistry — Wed, 30 Dec 2009 01:10:41 +0100

(Source: Annals of Clinical Biochemistry)

Concurrent Addison's disease and silent thyroiditis

Internal Medicine Journal — Wed, 30 Dec 2009 00:00:00 +0100

(Source: Internal Medicine Journal)

Subsequent Autoimmune or Related Disease in Asthma Patients: Clustering of Diseases or Medical Care?

Annals of Epidemiology — Mon, 28 Dec 2009 00:00:00 +0100

Conclusions: Hospitalized asthma patients presented with a number of subsequent autoimmune and related diseases. Although we were unable to exclude the effects of environmental factors, the data suggest that shared genetic factors or gene-environment interactions may explain coexistence of some of these diseases. (Source: Annals of Epidemiology)

Transient adrenal insufficiency in the premature newborn

Current Opinion in Endocrinology, Diabetes and Obesity — Thu, 24 Dec 2009 14:09:23 +0100

Purpose of review: Relative adrenal insufficiency is a controversial phenomenon described in adults and children with critical illness, especially septic shock. In the past 2 decades, relative adrenal insufficiency has also been reported in the critically ill premature as well as term newborn. The present study will review the initial and more recent studies addressing adrenal insufficiency in the premature infant. Recent findings: Studies suggest that 'relative adrenal insufficiency' is a contributing factor to hemodynamic instability in the sick preterm newborn. Many ill preterm newborns have inappropriately low serum cortisol concentrations and respond to steroid administration. Adrenal insufficiency is transient and likely reflects normal adrenal physiology at younger gestational ages...

Validated LC-MS/MS assay for the quantitative determination of vardenafil in human plasma and its application to a pharmacokinetic study.

Biomedical Chromatography : BMC — Wed, 23 Dec 2009 00:00:00 +0100

Authors: Lake ST, Altman PM, Vaisman J, Addison RS A sensitive high-performance liquid chromatography-tandem mass spectrometric (HPLC-MS/MS) assay has been developed for the quantitative analysis of vardenafil in human plasma. Vardenafil and the internal standard, alprazolam, were extracted from 0.2 mL aliquots of alkalinized plasma by a single solvent extraction into hexane : dichloromethane. Reversed-phase chromatographic separation was affected by gradient elution with mobile phases consisting of 10 mM ammonium formate pH 7.0 (solvent A) and methanol (100%, solvent B), delivered at a flow rate of 0.4 mL/min. The analytes were detected by using an electrospray ion source on a 4000 QTrap triple quadrupole mass spectrometer operating in positive ionization mode. The mass transitions we...

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API Software buys Texas software firm

bizjournals.com Health Care:Health Insurance headlines — Tue, 22 Dec 2009 17:15:22 +0100

API Healthcare, a Hartford provider of human capital management technology to the health care industry, said Tuesday it has acquired Clearview Staffing Software Inc., an Addison, Texas, provider of health care temp scheduling software. (Source: bizjournals.com Health Care:Health Insurance headlines)

API Software buys Texas software firm

bizjournals.com Health Care:Biotechnology headlines — Tue, 22 Dec 2009 17:15:22 +0100

API Healthcare buys Texas software firm

bizjournals.com Health Care:Biotechnology headlines — Tue, 22 Dec 2009 17:15:22 +0100

API Healthcare buys Texas software firm

bizjournals.com Health Care:Hospitals headlines — Tue, 22 Dec 2009 17:15:22 +0100

Precipitation and selective extraction of human serum endogenous peptides analysis by quadrupole time-of-flight mass spectrometry reveals posttranslational modifications and low-abundance peptides.

Analytical and Bioanalytical Chemistry — Tue, 22 Dec 2009 00:00:00 +0100

Authors: Williams D, Ackloo S, Zhu P, Bowden P, Evans KR, Addison CL, Lock C, Marshall JG The endogenous peptides of human serum may have regulatory functions, have been associated with physiological states, and their modifications may reveal some mechanisms of disease. In order to correlate levels of specific peptides with disease alongside internal standards, the polypeptides must first be reliably extracted and identified. Endogenous blood peptides can be effectively enriched by precipitation of the serum with organic solvents followed by selective extraction of peptides using aqueous solutions modified with organic solvents. Polypeptides on filter paper were assayed with Coomasie brilliant blue binding. The polypeptides were resolved by detergent tricine polyacrylamide electrophore...

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Serum macrophage migration inhibitory factor reflects adrenal function in hypothalamo-pituitary-adrenal axis in septic patients: an observational study

BMC Infectious Diseases — Mon, 21 Dec 2009 00:00:00 +0100

Conclusions: The inverse correlation between serum MIF and delta max cortisol or the delta max cortisol/albumin ratio suggests that high serum MIF reflects an insufficient adrenal response in the HPA axis. Serum MIF could be a valuable clinical marker of adrenal insufficiency in sepsis patients. (Source: BMC Infectious Diseases)

Supplemental perioperative steroids for surgical patients with adrenal insufficiency

NeLM - Drug Specific Reviews — Fri, 18 Dec 2009 00:00:00 +0100

Source: Cochrane Library Area: Evidence > Drug Specific Reviews Background Adrenal crisis is a life threatening condition which can be induced by stress during surgery in patients with adrenal insufficiency. This may be prevented by perioperative administration of high doses of steroids. There is disagreement on whether supplemental perioperative steroids are required and, when administered, on the amount and frequency of doses. Objectives To assess whether it is necessary to administer supplemental perioperative steroids in adult patients on maintenance doses of glucocorticoids because of adrenal insufficiency. Search strategy We searched the Cochrane Central Register of Controlled Trials (CENTRAL) (The Cochrane Library 2009, Issue 1); MEDLINE (1966 to January 2009)...

Lingual hyperpigmentation from pegylated interferon and ribavirin treatment of hepatitis C

Journal of the American Academy of Dermatology — Thu, 17 Dec 2009 14:02:47 +0100

We report a woman who developed lingual hyperpigmentation secondary to ribavirin and pegylated interferon (IFN) for the treatment of hepatitis C virus (HCV). A 36-year-old white woman with a history of HCV and psoriasis complained of sensitivity and slight discomfort associated with increased pigmentation on her tongue for the previous 2 months. Upon examination, she had dark gray macules on the bilateral dorsolateral surfaces of her tongue (). She did not have any oral erosions or any other nail or cutaneous findings. The only new medications that she had begun in close temporal proximity to the onset of the discomfort were peginterferon alfa-2b (120 μg injected subcutaneously once weekly) and ribavirin (400 mg twice daily), both of which were started 4 months prior to presentation. She ...

Development of a Disease-Specific Quality of Life Questionnaire in Addison's Disease.

The Journal of Clinical Endocrinology and Metabolism — Wed, 16 Dec 2009 00:00:00 +0100

Conclusions: We envisage AddiQoL having utility in trials of hormone replacement and management of patients with Addison's disease, analogous to similar questionnaires in GH deficiency (AGHDA) and acromegaly (AcroQoL). PMID: 20016050 [PubMed - as supplied by publisher] (Source: The Journal of Clinical Endocrinology and Metabolism)

Association of the CYP27B1 C(−1260)A Polymorphism with Autoimmune Addison's Disease

Experimental and Clinical Endocrinology and Diabetes — Sat, 12 Dec 2009 14:06:48 +0100

In conclusion, this study confirms the association of the C(−1260)A polymorphism with AAD, whereas the contribution of G7146A seems less likely.[...]© J. A. Barth Verlag in Georg Thieme Verlag KG Stuttgart · New YorkGet connected:Table of contents | Abstract | Full text (Source: Experimental and Clinical Endocrinology and Diabetes)

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Jane Austen 'died of tuberculosis not hormonal disorder' (Daily Telegraph, 1 December 2009)

Society for Endocrinology — Fri, 11 Dec 2009 12:00:00 +0100

Katherine White from the Addison's Disease Self Help Group (ADSHG) has written about Jane Austen's condition in the Medical Humanities journal and concluded that her symptoms were not indicative of Addison's disease as previously thought. Full articleADSHG website (Source: Society for Endocrinology)

Hedgehog signaling: Endocrine gland development and function

American Journal of Medical Genetics Part A — Thu, 10 Dec 2009 00:00:00 +0100

The role of hedgehog signaling is analyzed in relation to the developing endocrine glands: pituitary, ovary, testis, adrenal cortex, pancreas, prostate, and epiphyseal growth. Experimental and pathological correlates of these organs are also discussed. The second section addresses a number of topics. First, the pituitary gland, no matter how hypoplastic, is present in most cases of human holoprosencephaly, unlike animals in which it is always said to be absent. The difference appears to be that animal mutations and teratogenic models involve both copies of the gene in question, whereas in humans the condition is most commonly heterozygous. Second, tests of endocrine function are not reported with great frequency, and an early demise in severe cases of holoprosencephaly accounts for this tr...

Diagnostic utility of the glucagon stimulation test (GST) in comparison to the insulin tolerance test (ITT) in patients following pituitary surgery.

European Journal of Endocrinology — Tue, 08 Dec 2009 00:00:00 +0100

CONCLUSION: In our prospective series of patients with pituitary disease, GST is a potential alternative test for assessment of GH reserve, but poor for ACTH reserve. Test-specific cut-offs should be applied to avoid misinterpretation. PMID: 19996199 [PubMed - as supplied by publisher] (Source: European Journal of Endocrinology)

Pitfalls in the Diagnosis of Central Adrenal Insufficiency in Children.

Endocrine Development — Sat, 05 Dec 2009 18:58:26 +0100

Authors: Kazlauskaite R, Maghnie M The diagnosis of central adrenal insufficiency relies heavily on laboratory testing of cortisol levels in the systemic circulation. The lack of cortisol assay standardization challenges the reliability of dynamic tests of the hypothalamic-pituitary adrenal axis. Although the insulin-induced hypoglycemia or metyrapone tests remain the accepted standards for evaluating central adrenal insufficiency in children their associated risks and inconvenience make them unattractive for routine use. Corticotropin testing is an effective first step to evaluate for chronic central adrenal insufficiency for children older than 2 years who are ambulatory, have normal sleep-wake cycle and normal serum protein levels. The low-dose (1 mug) corticotropin test may be supe...

Nine Lessons and Carols for Godless People

Nature Network London - Upcoming Events — Fri, 04 Dec 2009 14:25:46 +0100

Tickets Still Available for the 20th December A night like no other this Christmas, a fractured variety version of the Royal Institute lectures mixing acclaimed evolutionary biologists with multi award-winning comedians and musicians Richard Dawkins, Dara O’Briain, Al Murray(The Pub Landlord) singing, Baba Brinkman’s Rap Guide to Evolution, Barry Cryer & Ronnie Golden, Simon Singh, Richard Herring, Ben Goldacre, Brian Cox, Chris Addison, Robyn Hitchcock, Mark Steel, John Gordillo, Martin White’s Mystery Fax Machine Orchestra, The BHA Choir, all hosted by multi-award winning comedian Robin Ince In association with the Rationalist Association and the Mustard Seed Secular School in Uganda. For more info click "here":http://newhumanist.org.uk/ra Venue: Hamm...

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The rs1990760 polymorphism within the IFIH1 locus is not associated with Graves' disease, Hashimoto's thyroiditis and Addison's disease

BMC Medical Genetics - Latest articles — Fri, 04 Dec 2009 00:00:00 +0100

Conclusion: In conclusion, our data suggest, no contribution from IFIH1 rs1990760 polymorphism to the pathogenesis of either Graves' disease, Hashimoto's thyroiditis or Addison's disease in our study populations. However, in order to exclude a possible influence of the studied polymorphism in specified subgroups within patients with autoimmune thyroid disease, further investigations in larger populations are needed. (Source: BMC Medical Genetics - Latest articles)

High-Calorie Glucose-Rich Food Attenuates Neuroglycopenic Symptoms in Patients with Addison's Disease.

The Journal of Clinical Endocrinology and Metabolism — Fri, 04 Dec 2009 00:00:00 +0100

Conclusions: High-calorie comfort food reduces symptoms of neuroglycopenia in Addison patients, suggesting that Addison's disease is associated with a deficit in cerebral energy supply that can partly be alleviated by intake of palatable food. It will be important to investigate whether additional oral glucose supply may be helpful in improving patients' well-being. PMID: 19965917 [PubMed - as supplied by publisher] (Source: The Journal of Clinical Endocrinology and Metabolism)

Health News of the Day

Clinical Cases and Images — Thu, 03 Dec 2009 11:40:00 +0100

is a daily summary made from the selected links I post on Twitter. It is in a bullet points format with links to the original sources which include 350 RSS feeds that produce about 2,500 items per day:A Look at the Low-Carbohydrate Diet: Mice on high-fat, high-protein, low-carb diet developed atherosclerosis not associated with traditional risk factors http://bit.ly/4J1gBaStudy: Current EMR systems might modestly improve measures of quality but do not reduce costs (PDF) http://bit.ly/6QgH7bJane Austen may have died of tuberculosis rather than Addison's disease, as has long been believed http://bit.ly/6tRNu3Low birth weight associated with increased risk of CKD in later life http://bit.ly/6sCtTBShould Vending Machines Post Calorie Counts? http://bit.ly/55hWUM - They should."You become marr...

What really killed Jane Austen?

CNN.com - Health — Wed, 02 Dec 2009 22:37:15 +0100

A woman who's studied the writer's letters believes she didn't die of Addison's disease. (Source: CNN.com - Health)

New Light on Jane Austen's Final Chapter

Forbes.com Health News — Wed, 02 Dec 2009 14:00:00 +0100

Novelist's symptoms point to death from TB, not Addison's, researcher says (Source: Forbes.com Health News)

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A Case of Human Intramuscular Adrenal Gland Transplantation as a Cure for Chronic Adrenal Insufficiency

American Journal of Transplantation — Wed, 02 Dec 2009 00:00:00 +0100

We describe here the case of a 5-year-old girl with renal failure who had adrenal insufficiency following fulminant meningococcemia that led to requirements for both stress-dose steroid and mineralocorticoid replacement. Ten months after the onset of her disease, she received a simultaneous renal and adrenal gland transplant from her mother. The adrenal gland allograft was morselized into 1 mm3 segments and implanted into three 2 cm pockets created in her rectus abdominis muscle. Three years after surgery, her allograft remains fully functional, responding well to adrenocorticotropin hormone stimulation and the patient does not require any steroid or mineralcorticoid supplementation. We believe this case represents the first description of successful functional intramuscular adrenal allogr...

[Fatigue and anemia.]

Praxis — Wed, 02 Dec 2009 00:00:00 +0100

Authors: Ivanova K, Zeller A We herein report on an 80-year old male patient with a history of muscle weakness, fatigue and weight loss since several months. Because of a pathologic synacthen test in combination with decreased levels of ACTH, we diagnosed a secondary chronic adrenal insufficiency. Because of a normochromic, normocytic, and hypoproliferativ anemia, bone marrow puncture was performed, showing an anemia of chronic disease. We initiated Hydrocortison and anemia and patients' symptoms were fully reconstituted. PMID: 19953473 [PubMed - in process] (Source: Praxis)

Epidemiology of adrenal crisis in chronic adrenal insufficiency - the need for new prevention strategies.

European Journal of Endocrinology — Wed, 02 Dec 2009 00:00:00 +0100

Conclusion: AC occurs in a substantial proportion of patients with chronic AI, mainly triggered by infectious disease. Only a limited number of risk factors suitable for targeting prevention of AC were identified. These findings indicate the need for new concepts of crisis prevention in patients with AI. PMID: 19955259 [PubMed - as supplied by publisher] (Source: European Journal of Endocrinology)

Addison Disease

Pediatrics in Review — Tue, 01 Dec 2009 16:01:41 +0100

(Source: Pediatrics in Review)

Adrenal Insufficiency in the Pediatric Emergency Department

Clinical Pediatric Emergency Medicine — Tue, 01 Dec 2009 00:00:00 +0100

Adrenal insufficiency is an important and potentially life-threatening condition that may present to the emergency department. Seven clinical scenarios of adrenal insufficiency that the emergency physician should be able to recognize and confidently manage are reviewed. Epidemiology and mortality, etiology, and pathophysiology are addressed. Clinical presentation, diagnosis, and management of acute and chronic adrenal disease are also covered. In particular, adrenal suppression due to exogenous steroid use, adrenal suppression in septic shock, and adrenal suppression associated with etomidate are reviewed. (Source: Clinical Pediatric Emergency Medicine)

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Jane Austen and Addison's disease: an unconvincing diagnosis

Medical Humanities — Mon, 30 Nov 2009 18:48:26 +0100

Jane Austen’s letters describe a two-year deterioration into bed-ridden exhaustion, with unusual colouring, bilious attacks and rheumatic pains. In 1964, Zachary Cope postulated tubercular Addison’s to explain her symptoms and her relatively pain-free illness. Literary scholars later countered this posthumous diagnosis on grounds that are not well substantiated, while medical authors supported his conclusion. Important symptoms reported by contemporary Addison’s patients—mental confusion, generalised pain and suffering, weight loss and anorexia—are absent from Jane Austen’s letters. Thus, by listening to the patient’s perspective, we can conclude it is unlikely that Addison’s disease caused Jane Austen’s demise. Disseminated bovine tube...

Diagnosis of Adrenal Insufficiency Using the GHRP-6 Test: Comparison with the Insulin Tolerance Test in Patients with Hypothalamic-Pituitary-Adrenal Disease

Hormone and Metabolic Research — Sat, 28 Nov 2009 02:44:22 +0100

Horm Metab ResDOI: 10.1055/s-0029-1243184AbstractThe insulin tolerance test (ITT) is considered the gold standard for the diagnosis of adrenal insufficiency (AI). However, the test is unpleasant to perform and has the risk of serious complications. We therefore evaluated the clinical applicability of GHRP6, which is a known activator of the hypothalamic-pituitary-adrenal (HPA) axis, to test for AI. For this purpose a comparative clinical study was designed. Forty-nine patients with suspected dysfunction of the HPA axis and 20 healthy controls were enrolled. The ITT was performed in patients, and GHRP6 (1 μg/kg) testing in patients and controls. Serum cortisol over 90 min after GHRP6, in comparison to the ITT, was the main outcome measure. Thirty-one patients had a peak cor...

Surgery in the Patient with Endocrine Dysfunction

Anesthesiology Clinics — Fri, 27 Nov 2009 13:42:06 +0100

This article focuses on 4 of the most common endocrinopathies: diabetes mellitus, hyperthyroidism, hypothyroidism, and adrenal insufficiency. Perioperative challenges in patients presenting with pheochromocytoma are also discussed. (Source: Anesthesiology Clinics)

Perioperative Steroid Dosing in Patients Receiving Chronic Oral Steroids, Undergoing Outpatient Hand Surgery

The Journal of Hand Surgery — Fri, 27 Nov 2009 00:00:00 +0100

Historically, patients who are receiving oral glucocorticoids and undergo surgical procedures were administered supplemental intravenous steroids during the perioperative period. This practice remains controversial, however, for less invasive outpatient procedures such as those performed by hand surgeons on a routine basis. To date, there are no evidence-based treatment guidelines that provide firm recommendations for the administration of perioperative steroids; rather, present management is based on case reports and observational studies. In this article, we discuss the pathophysiology behind acute adrenal insufficiency, the stress response and its suppression, patients who may be at risk, the effect of inhaled and topical steroids, and published data that guide our practice concerning t...

Primary adrenal failure due to viral infection in an infant

European Journal of Pediatrics — Thu, 19 Nov 2009 20:00:50 +0100

Abstract Acquired primary adrenal insufficiency is a rare disorder in childhood. The most common cause is autoimmune adrenalitis, especially as a part of polyendocrinopathy syndromes. Impaired adrenal function is seen in patients infected with HIV. In adult patients with AIDS, cytomegalovirus (CMV)-associated adrenal insufficiency is a well-known condition, whereas CMV infection as a causing adrenal insufficiency in children is very rare. Here, we report an infant with transient adrenal insufficiency associated with CMV infection but without HIV. She was treated successfully with steroid replacement and ganciclovir. Early diagnosis and treatment is lifesaving in these patients. Content Type Journal ArticleCategory Short ReportDOI 10.1007/s00431-009-1103-0Authors Leyla ...

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International Society for Heart & Lung Transplantation Research Fellowship

ScanGrants feed — Wed, 18 Nov 2009 00:00:00 +0100

International Society for Heart & Lung Transplantation Research Fellowship ISHLT issues up to three Research Fellowship Awards annually. Each award is in the amount of $40,000. The funding period is for one year. Financial Support The 2010 Awards Program is supported by educational grants from Novartis, Roche, Astellas, XDX and Gilead. Eligibility Requirements 1. The applicant or the applicant's chief of staff/research project director must be a member of the ISHLT in good standing at the time of application and throughout the period of funding. 2. The applicant must be in a clinical or post-doctoral training program and not yet have attained Board certification/accreditation/faculty level appointment (Assistant Professor equivalent or above)/salaried senior staff position (or equiva...

International Society for Heart & Lung Transplantation Nursing and Social Sciences Research Grant

ScanGrants feed — Wed, 18 Nov 2009 00:00:00 +0100

International Society for Heart & Lung Transplantation Nursing and Social Sciences Research Grant ISHLT issues at least one Nursing and Social Sciences Grant annually in an amount of up to $12,000. The funding period is for one year. Purpose The purpose of this award is to encourage qualified nurses, social scientists and other health care professionals to conduct research related to the areas of end-stage heart and lung disease and transplantation. This award is designated for non-physician health professionals. Eligibility Criteria 1. The PI must be a non-physician member of ISHLT and the Council on Nursing, Health Science & Allied Health. 2. The PI must have at least a bachelor's degree. 3. The PI may be a new or established researcher. 4. The PI must have either direct clinic...

International Society for Heart & Lung Transplantation 2010 Branislav Radovancevic Memorial Fellowship Grant

ScanGrants feed — Wed, 18 Nov 2009 00:00:00 +0100

International Society for Heart & Lung Transplantation 2010 Branislav Radovancevic Memorial Fellowship Grant This Award is issued annually in the amount of $75,000. The funding period is for one year. Purpose The purpose of this award is to encourage scholarly clinical work in mechanical circulatory support, particularly in emerging countries, to facilitate scientific exchange regarding MCS, and to provided advanced scholarly clinical training in mechanical circulatory support and total artificial heart therapy. Dr. Radovancevic, a long-time member of ISHLT, was devoted to the encouragement of scientific collaboration with Eastern European physicians and scientists. This award is intended to encourage others to continue his efforts. Funding for this award is provided by Thoratec in me...

Clinical profile of adrenoleukodysrophy

Indian Journal of Pediatrics — Wed, 11 Nov 2009 19:13:58 +0100

Abstract X-linked Adrenoleukodystrophy (ALD) is the most common of the peroxisomal disorder and is associated with functional defect of the very long chain fatty acid (VLCFA) oxidation leading to the accumulation of VLCFA in the white matter and adrenal cortex. Retrospective evaluation of medical records of ALD patients were carried out. In all the 5 patients the duration of the symptoms varied from 1–7 years. Most of them presented with Addisonian crisis (4/5) and hyperpigmentation (5/5), white half of them (3/5) had neurological symptoms. All patients had biochemical evidence of the adrenal insufficiency. All siblings of patients should be screened for the possibility of ALD with VLCFA. Content Type Journal ArticleCategory Clinical BriefDOI 10.1007/s12098-009-0220-0A...

Megestrol: Adrenal insufficiency in an elderly patient: case report

Reactions — Wed, 11 Nov 2009 14:08:04 +0100

(Source: Reactions)

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Long-Term Safety of Recombinant Human Growth Hormone in Children.

The Journal of Clinical Endocrinology and Metabolism — Wed, 11 Nov 2009 00:00:00 +0100

Conclusion: After more than 20 yr, leukemia, a major safety issue initially believed associated with GH, has not been confirmed, but other signals, including risk of second malignancies in patients previously treated with irradiation, have been detected or confirmed through the NCGS. These data further clarify the events associated with rhGH and, although confirming a favorable overall safety profile, they also highlight specific populations at potential risk. PMID: 19906787 [PubMed - as supplied by publisher] (Source: The Journal of Clinical Endocrinology and Metabolism)

Isolated Addison's is unlikely to be caused by mutations in MC2R, MRAP or StAR, three genes responsible for familial glucocorticoid deficiency.

European Journal of Endocrinology — Tue, 10 Nov 2009 00:00:00 +0100

Conclusions FGD does not appear be underdiagnosed in the AD population. However, in ~50% of patients with FGD no genetic cause has yet been identified and it is possible that the other, as yet unidentified genes giving rise to FGD maybe implicated in AD. PMID: 19903795 [PubMed - as supplied by publisher] (Source: European Journal of Endocrinology)

Hypoglycaemic seizure and neonatal acute adrenal insufficiency after maternal exposure to prednisone during pregnancy: a case report

European Journal of Pediatrics — Mon, 09 Nov 2009 19:14:00 +0100

We report a case of AAI following maternal exposure to moderate doses of corticosteroids during pregnancy. The neonate, born at term, presented with hypoglycaemia complicated by seizures. The AAI was confirmed by endocrine tests and after exclusion of differential diagnoses. Early diagnosis and treatment led to full recovery. Content Type Journal ArticleCategory Short ReportDOI 10.1007/s00431-009-1095-9Authors Pierre-Jean Saulnier, CHU de Poitiers Department of Clinical Pharmacology and Regional Center of Pharmacovigilance Poitiers FranceXavier Piguel, CHU de Poitiers Department of Endocrinology and Diabetology Poitiers FranceMarie Christine Perault-Pochat, CHU de Poitiers Department of Clinical Pharmacology and Regional Center of Pharmacovigilance Poitiers FranceCassandra Csizmadi...

Identification of a novel mutation in DAX1/NR0B1A gene in two siblings with severe clinical presentation of adrenal hypoplasia congenita

Arquivos Brasileiros de Endocrinologia e Metabologia — Fri, 06 Nov 2009 14:11:07 +0100

CONCLUSION: A novel frameshift mutation of DAX1 gene, which established the molecular etiology of the AHC in the siblings, was identified. Obtaining a precise genetic diagnosis of this adrenal disorder, which, sometimes, cannot be confirmed only by clinical aspects, may have important implications for the long-term management of the disease.OBJETIVO: Pesquisar mutações no gene DAX1/NR0B1A em dois irmãos com suspeita de hipoplasia adrenal congênita (HAC), rara doença potencialmente fatal, para estabelecer sua etiologia molecular. RELATO DOS CASOS: São apresentados os relatos de dois irmãos com síndrome perdedora de sal no período neonatal que receberam terapia de reposição hormonal para insuficiência adrenal primária. O diagnóstico de HAC foi suspeitado porque as crianças man...

Serum total cortisol and free cortisol index give different information regarding the hypothalamus-pituitary-adrenal axis reserve in patients with liver impairment

Annals of Clinical Biochemistry — Wed, 04 Nov 2009 19:16:54 +0100

Conclusion When total cortisol alone is used to interpret SST in patients with liver impairment, 46% may have been classified as having adrenal insufficiency because of low CBG. FCI may be better for the evaluation of HPA axis insufficiency in patients with liver impairment. (Source: Annals of Clinical Biochemistry)

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An unusual case of profound hyponatraemia and bilateral adrenal calcifications

Annals of Clinical Biochemistry — Wed, 04 Nov 2009 19:16:54 +0100

We report a case of a 65-year-old lady who presented with acute confusion and profound hyponatraemia (plasma sodium of 97 mmol/L). Five years earlier she had developed sepsis and was found to have hyponatraemia, thought to be due to syndrome of inappropriate antidiuretic hormone secretion. The patient was lost to follow-up. The patient was covered with steroids and investigations confirmed primary adrenal failure with flat response of cortisol to adrenocorticotropic hormone (ACTH) stimulation and very high level of ACTH. Adrenal auto-antibodies were negative and a computed tomography of the adrenals showed bilateral adrenal calcifications, suggestive of previous haemorrhage or infarction. Bilateral adrenal calcification due to haemorrhage/infarction usually does not present with severe hyp...

T Cell Responses to Steroid Cytochrome P450 21-Hydroxylase in Patients with Autoimmune Primary Adrenal Insufficiency.

The Journal of Clinical Endocrinology and Metabolism — Wed, 04 Nov 2009 00:00:00 +0100

Conclusion: Patients with autoimmune Addison's disease have circulating 21OH-specific T cells, with amino acids 342-361 of 21OH possibly constituting a disease-specific epitope presented by HLA-DRB1*0404. PMID: 19890026 [PubMed - as supplied by publisher] (Source: The Journal of Clinical Endocrinology and Metabolism)

Cytotoxic T lymphocyte antigen-4 Ala17 polymorphism is a genetic marker of autoimmune adrenal insufficiency: Italian association study and meta-analysis of European studies.

European Journal of Endocrinology — Mon, 02 Nov 2009 00:00:00 +0100

Conclusions: The CTLA4 +49 polymorphism is strongly associated with genetic risk for AAD, independentenly from the well known association with HLA class II genes. PMID: 19884265 [PubMed - as supplied by publisher] (Source: European Journal of Endocrinology)

GH replacement in adults: interactions with other pituitary hormone deficiencies and replacement therapies.

European Journal of Endocrinology — Sun, 01 Nov 2009 00:00:00 +0100

Authors: Filipsson H, Johannsson G Severe GH deficiency (GHD) in adults has been described as a clinical entity. However, some of the features associated with GHD could be due to unphysiological and inadequate replacement of other pituitary hormone deficiencies. This may be true for glucocorticoid replacement that lacks a biomarker making dose titration and monitoring difficult. Moreover, oral estrogen replacement therapy decreases IGF1 levels compared with the transdermal route, which attenuates the responsiveness to GH replacement therapy in women. In addition, in untreated female hypogonadism, oral estrogen may augment the features associated with GHD in adult women. Important interactions between the hormones used for replacing pituitary hormone deficiency occur. Introducing GH rep...

Type 1 diabetes and autoimmune polyglandular syndrome: a clinical review.

The Netherlands Journal of Medicine — Sun, 01 Nov 2009 00:00:00 +0100

Authors: Van den Driessche A, Eenkhoorn V, Van Gaal L, De Block C Type 1 diabetes mellitus (T1DM) results from autoimmune destruction of insulin-producing β; cells and is characterised by the presence of insulitis and β;-cell autoantibodies. Up to one third of patients develop an autoimmune polyglandular syndrome. Fifteen to 30% of T1DM subjects have autoimmune thyroid disease (Hashimoto's or Graves' disease), 5 to 10% are diagnosed with autoimmune gastritis and/or pernicious anaemia (AIG /PA), 4 to 9% present with coeliac disease (CD), 0.5% have Addison's disease (AD), and 2 to 10% show vitiligo. These diseases are characterised by the presence of autoantibodies against thyroid peroxidase (for Hashimoto's thyroiditis), TSH receptor (for Graves' diseas...

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Whole-body bone scintigraphy provides a measure of the total-body burden of osteoarthritis for the purpose of systemic biomarker validation

Arthritis and Rheumatism — Thu, 29 Oct 2009 00:00:00 +0100

To evaluate the association of serum and synovial fluid cartilage oligomeric matrix protein (COMP) with systemic and local measures of osteoarthritis (OA) activity by bone scintigraphy.Samples of serum and knee joint synovial fluid (275 knees) were obtained from 159 patients with symptomatic OA of at least 1 knee. Bone scintigraphy using 99mTc-labeled methylene diphosphonate was performed, and early-phase knee scans and late-phase whole-body bone scans of 15 additional joint sites were scored semiquantitatively. To control for within-subject correlations of knee data, generalized linear modeling was used in the correlation of the bone scan scores with the COMP levels. Principal components analysis was used to explore the contribution of each joint site to the variance in serum COMP levels....

Words of Wisdom Wednesday: Notable Quotables

Psychology Today Parenting Center — Wed, 28 Oct 2009 23:17:35 +0100

I couldn't resist posting some of my favorite quotes about the dynamic of the father-daughter relationship. What are some of your favorites? I'd love to hear them!"Certain is it that there is no kind of affection so purely angelic as of a father to a daughter. In love to our wives there is desire; to our sons, ambition; but to our daughters there is something which there are no words to express." --Joseph Addison"To a father growing old nothing is dearer than a daughter." --Euripides"He opened the jar of pickles when no one else could. He was the only one in the house who wasn't afraid to into the basement by himself. He cut himself shaving, but no one kissed it or got excited about it. It was understood when it rained, he got the car and brought it around to the door. Wh...

Corticomedullary mixed tumor of the adrenal gland.

Annales d'Endocrinologie — Wed, 28 Oct 2009 00:00:00 +0100

Authors: Trimeche Ajmi S, Chadli Chaieb M, Mokni M, Braham R, Ach K, Maaroufi A, Chaieb L A 34-year-old woman presented with weight gain, hirsutism, recent hypertension and secondary amenorrhea. Laboratory findings showed hypokalemia, elevated cortisol and androgen levels with normal urine metanephrines and normal aldosteronemia. Abdominal magnetic resonance imaging showed a right heterogeneous adrenal mass measuring 4x6cm with mixed component of fat and adrenal tissue suggesting corticosurrenaloma. After right adrenalectomy, blood pressure normalized and hypokalemia resolved. In the postoperative course, the patient presented adrenal insufficiency treated with hydrocortisone hemisuccinate. Histological examination showed a single tumor mass composed of an admixed population of adrenal...

Clinical, Immunological, and Genetic Features of Autoimmune Primary Adrenal Insufficiency: Observations from a Norwegian Registry.

The Journal of Clinical Endocrinology and Metabolism — Mon, 26 Oct 2009 00:00:00 +0100

Conclusions: AD is almost exclusively autoimmune, with high autoimmune comorbidity. Both anti-21-hydroxylase antibodies and HLA class II can be clinically relevant predictors of AD. HRQoL is reduced, especially among diabetes patients, whereas thyroid disease did not have an impact on HRQoL. Treatment modalities that improve HRQoL are needed. PMID: 19858318 [PubMed - as supplied by publisher] (Source: The Journal of Clinical Endocrinology and Metabolism)

Programmed Death Ligand 1 (PD-L1) Gene Variants Contribute to Autoimmune Addison's Disease and Graves' Disease Susceptibility.

The Journal of Clinical Endocrinology and Metabolism — Thu, 22 Oct 2009 00:00:00 +0100

Conclusions: We confirm the role of PD-L1 variants in GD susceptibility and extend these findings to demonstrate association in two Northern European patient cohorts with AAD. PD-L1 joins the growing number of known susceptibility loci exerting modest effects in these autoimmune disorders. PMID: 19850680 [PubMed - as supplied by publisher] (Source: The Journal of Clinical Endocrinology and Metabolism)

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Pre-Operative Chest X-Ray (CXR) In Breast Cancer Patients - Is It Necessary?

European Journal of Surgical Oncology — Fri, 16 Oct 2009 12:47:00 +0100

Introducation: There is general consensus that routine pre-operative staging in breast cancer patients is unnecessary. In majority of cases, chest X-rays continue to be performed prior to surgery in centres in the UK. If it were demonstrated that this practice was ineffective, units would be able to reduce costs, time and radiation exposure to patients. The aim of this study was to determine detection rates of pulmonary metastases, on routine CXR, in patients undergoing curative surgery for breast cancer. (Source: European Journal of Surgical Oncology)

[Correspondence] Etomidate versus ketamine for sedation in acutely ill patients

LANCET — Thu, 08 Oct 2009 23:00:00 +0100

In their study of sedative drugs for emergency intubation, Patricia Jabre and colleagues conclude that etomidate is associated with an increased incidence of adrenal insufficiency, defined as a random serum cortisol concentration of less than 276 nmol/L or an increase in serum cortisol concentration of less than 250 nmol/L after adrenocorticotropin stimulation. This conclusion might not be warranted, since current clinical testing for adrenocortical function in critically ill patients is unreliable. (Source: LANCET)

[Correspondence] Etomidate versus ketamine for sedation in acutely ill patients – Authors' reply

LANCET — Thu, 08 Oct 2009 23:00:00 +0100

Nicolas Mongardon, Mervyn Singer, and David Kaufman all address questions related to the adrenal insufficiency seen in our trial. We agree with Mongardon and Singer that it is important to note patients who received corticosteroids during follow-up. 73 of 469 patients analysed received corticosteroids after admission to the intensive-care unit. 28-day mortality in the etomidate group did not differ significantly between steroid-treated patients and non-steroid-treated patients (13/42 [31%] vs 68/192 [35%]; p=0·58). These results are in accordance with those reported by Cuthbertson and colleagues in their CORTICUS substudy of hydrocortisone in septic shock. (Source: LANCET)

Homozygosity of the Polymorphism MICA5.1 Identifies Extreme Risk of Progression to Overt Adrenal Insufficiency among 21-Hydroxylase Antibody-Positive Patients with Type 1 Diabetes.

The Journal of Clinical Endocrinology and Metabolism — Thu, 08 Oct 2009 23:00:00 +0100

Conclusions: Identifying extreme risk should facilitate monitoring of progression from 21OH antibody positivity to overt AD. The HLA-DR3/0404 genotype defines high-risk subjects for adrenal autoimmunity. MICA5.1/5.1 may define those at highest risk for progression to overt AD, a feature unique to AD and distinct from T1D. PMID: 19820007 [PubMed - as supplied by publisher] (Source: The Journal of Clinical Endocrinology and Metabolism)

Social, organizational, and contextual characteristics of clinical decision support systems for intensive insulin therapy: A literature review and case study

International Journal of Medical Informatics — Thu, 08 Oct 2009 00:00:00 +0100

Conclusion: This paper addresses a gap in the literature concerning the social, organizational, and contextual characteristics of CDSS in general and for intensive insulin therapy specifically. Additionally, this paper identifies areas for future research to define optimal computer-based IIT process execution: the frequency and effect of manual data entry error of blood glucose values, the frequency and effect of nurse overrides of CDSS insulin dosing recommendations, and comprehensive ethnographic study of CDSS for IIT. (Source: International Journal of Medical Informatics)

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Oxygen inhibition and incremental layer bond strengths of resin composites

Dental Materials — Mon, 05 Oct 2009 19:02:55 +0100

Abstract: Objectives: When dentists light cure resin composite restorations in increments or after contouring the surface layer to shape with a hand instrument the surface layer is exposed to air during polymerization. The presence of an oxygen inhibited resin surface layer may impact on clinical performance. Conflicting data has been produced in vitro regarding this topic.Methods: To shed further light on this subject the current investigation assessed the thickness of the oxygen inhibited layer (OIL) and subsequent interfacial bond strength at various times post-cure of an “initial increment” for a range of experimental and commercial resin composites. The latter included conventional methacrylate-based composites and a novel low shrink Silorane resin chemistry product.Results: A dec...

Flexion contractures in secondary adrenal insufficiency

Clinical Rheumatology — Thu, 01 Oct 2009 05:48:05 +0100

We report the case of a 55-year-old male with flexion contractures of the hips and the knees due to an isolated adrenocorticotropin (ACTH) deficiency, a rare cause of secondary adrenal insufficiency. The presenting symptoms and signs, the laboratory investigations and the treatment are described. The case description is followed by a brief overview of ACTH deficiency and its symptoms and causes. Our case is then compared with other case reports available in the literature. The aetiology of the flexion contractures in adrenal insufficiency is still unclear. The authors want to draw attention to adrenal insufficiency as the cause of unexplained flexion contractures. Content Type Journal ArticleCategory Case ReportDOI 10.1007/s10067-009-1289-6Authors Jan Berger, ZNA Middelheim Depar...

Adrenal insufficiency in acute coronary syndrome.

Singapore Medical Journal — Thu, 01 Oct 2009 00:00:00 +0100

Conclusion: Utilising the LDT, adrenal insufficiency is present in 21.6 percent of patients admitted with ACS. However, this is not associated with any significant morbidity and mortality. PMID: 19907885 [PubMed - in process] (Source: Singapore Medical Journal)

Bilateral ossification of the auricles: an unusual entity and review of the literature

Head & Face Medicine — Wed, 30 Sep 2009 23:00:00 +0100

Conclusion: True auricular ossification is a quite rare clinical entity with unclear pathogenesis and one should have in mind that there is always the possibility of a serious co-existed disease like endocrinopathy. (Source: Head & Face Medicine)

Technical details influence the diagnostic accuracy of the 1 mcg ACTH stimulation test.

European Journal of Endocrinology — Wed, 30 Sep 2009 23:00:00 +0100

Conclusions: The high rate of abnormal results, especially in the afternoon, and loss of ACTH through tubing, suggest that morning testing and minimal tubing should be adopted to avoid an inappropriate diagnosis of adrenal insufficiency. Earlier time-points and standardized protocols would facilitate comparison of studies. PMID: 19797501 [PubMed - as supplied by publisher] (Source: European Journal of Endocrinology)

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Prevalence of occult adrenal insufficiency and the prognostic value of a short corticotropin stimulation test in patients with septic shock

Indian Journal of Critical Care Medicine — Sat, 26 Sep 2009 17:49:00 +0100

Conclusion:</b> A short corticotropin test using low-dose corticotropin (1 &#956;g) has a good prognostic value. High basal cortisol and a low increase in cortisol on corticotropin stimulation test are predictors of a poor outcome in patients with septic shock. (Source: Indian Journal of Critical Care Medicine)

Vendor Notebook - MEDSEEK delivers eHealth ecoSystem to N.C. healthcare company

Healthcare IT News — Fri, 25 Sep 2009 11:21:20 +0100

MEDSEEK, a Birmingham, Ala.-based provider of healthcare portal and connectivity solutions, has announced that Rex Healthcare, based in Raleigh, N.C., will implement the company’s comprehensive eHealth ecoSystem virtual community. Medicity, Inc., based in Salt Lake City, has announced that IASIS Healthcare has gone live with the Medicity Novo Grid connectivity solution in two (Florida and Utah) of its six healthcare regions, comprising 16 healthcare facilities. (Source: Healthcare IT News)

Disorders of Pigmentation

JDDG — Thu, 24 Sep 2009 23:00:00 +0100

Skin color is highly individual and the variations are controlled by numerous genes. The different skin colors result from the size and number of melanosomes and do not mirror the amount of melanocytes. Disorders of pigmentation can result from migration abnormalities of melanocytes from the neural crest to the skin during embryogenesis. In addition, impairment of melanosome transfer to the surrounding keratinocytes, an alteration in melanin synthesis and a defective degradation or removal of melanin may lead to abnormal skin pigmentation. Immunologic or toxic mediated destructions of melanocytes can end in pigmentation disorders. Disorders of pigmentation are classified in hypo- or hyperpigmentation which can occur as a genetic or acquired disease. They can manifest locally or diffuse. Co...

Hypopituitarism following traumatic brain injury: the prevalence is affected by the use of different dynamic tests and different normal values.

European Journal of Endocrinology — Thu, 24 Sep 2009 23:00:00 +0100

Conclusion: The reported variations in the prevalence rates of hypopituitarism after TBI are in part caused by differences in definitions, endocrine assessments of hypopituitarism and confounding factors. These methodological issues prohibit simple generalizations of results of original studies on TBI-associated hypopituitarism in the perspective of meta-analyses or reviews. PMID: 19783619 [PubMed - as supplied by publisher] (Source: European Journal of Endocrinology)

Pulmonary hypertension in a patient with Schmidt syndrome

The American Journal of Emergency Medicine — Thu, 24 Sep 2009 16:29:48 +0100

We describe a 26-year-old patient with long-standing autoimmune hypothyroidism. She was doing well until she developed Addisonian crisis accompanied by severe metabolic acidosis, hypoglycemia, hypomagnesemia, and hypokalemia. Subsequently she developed a life-threatening cardiac arrhythmia due to QT prolongation secondary to electrolyte imbalance. The association of autoimmune hypothyroidism and adrenal insufficiency in our patient suggests the diagnosis of autoimmune polyglandular syndrome type II or Schmidt syndrome. An echocardiography that was performed detected pulmonary hypertension without apparent cardiac or lung pathology. The association of pulmonary hypertension and Schmidt syndrome is rare and may be explained by a generalized immune activation leading to pulmonary endothelial ...

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ULURU Inc. Presents Data on Negative Pressure Related to Altrazeal(TM) at the Diabetic Limb Salvage Conference in Washington, D.C.

HSMN NewsFeed — Thu, 24 Sep 2009 15:56:21 +0100

ADDISON, Texas, Sept. 24 (HSMN NewsFeed) -- ULURU Inc. (NYSE Alternext: ULU) announced today the presentation of important clinical data and research at the 2009 Diabetic Limb Salvage (DLS) Conference in Washington, D.C. One poster presentation demonstra... Devices, Wound CareULURU, Altrazeal, Nanoflex (Source: HSMN NewsFeed)

Adrenal crisis in treated Addison's disease: a predictable but under-managed event.

European Journal of Endocrinology — Tue, 22 Sep 2009 23:00:00 +0100

Conclusion The endocrinologist has a responsibility to ensure that Addison's patients have adequate access to life-saving emergency injection materials and repeated, practical training sessions in how to use them, while the GP plays a vital role as in arranging prompt emergency admissions. PMID: 19776201 [PubMed - as supplied by publisher] (Source: European Journal of Endocrinology)

A Systematic Review and Meta-Analysis of Randomized Placebo-Controlled Trials of DHEA Treatment Effects on Quality of Life in Women with Adrenal Insufficiency.

The Journal of Clinical Endocrinology and Metabolism — Mon, 21 Sep 2009 23:00:00 +0100

Conclusions: DHEA may improve, in a small and perhaps trivial manner, HRQOL and depression in women with adrenal insufficiency. There was no significant effect of DHEA on anxiety and sexual well-being. The evidence appears insufficient to support the routine use of DHEA in women with adrenal insufficiency. PMID: 19773400 [PubMed - as supplied by publisher] (Source: The Journal of Clinical Endocrinology and Metabolism)

International Society for Magnetic Resonance in Medicine New Entrant Stipend Program

ScanGrants feed — Thu, 17 Sep 2009 23:00:00 +0100

Membership Application/Dues Payment Deadline: 3 December 2009New Entrant Stipend Application Deadline: 10 December 2009The ISMRM invites applications for stipends that encourage new entrants to research in areas of interest to the ISMRM. Each awardee will receive US$400 toward the costs of attending the Joint Annual Meeting ISMRM-ESMRMB 2010 in Stockholm, Sweden in May 2010.New This Year: 2009 ISMRM membership is required before application for a New Entrant stipend. If you are not a member, you will need to complete the membership application process on or before 3 December 2009 in order to apply for a New Entrant Stipend. If you are already a member, you will need to pay your 2010 membership dues on or before 3 December 2009. Who Should Apply? * Students, trainees, or researchers wh...

Factors Contributing to Hospitalization for Gastroparesis Exacerbations

Digestive Diseases and Sciences — Thu, 17 Sep 2009 12:37:33 +0100

Conclusions Poor glycemic control, infection, noncompliance with/intolerance of medications, and, perhaps, adrenal insufficiency were contributory factors leading to hospitalizations of gastroparetic patients. Hospitalized patients with gastroparesis exacerbations had elevated ESR and CRP levels. Although many patients with elevated inflammatory markers had evidence of infection, some did not. Assessment of inflammatory markers may help indicate those gastroparetic patients in whom a search for infection should be undertaken. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s10620-009-0975-1Authors Sesha S. Uppalapati, Temple University School of Medicine Gastroenterology Section, Department of Medicine Parkinson Pavilion, 8th Floor, 3401 North Broad...

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ULURU Inc. Presents Results of First Randomized Clinical Trial for Altrazeal(TM) at the Symposium on Advanced Wound Care (SAWC) Fall Meeting in Washington D.C.

HSMN NewsFeed — Wed, 16 Sep 2009 22:52:08 +0100

ADDISON, Texas, Sept. 16 (HSMN NewsFeed) -- ULURU Inc. (NYSE Alternext: ULU) announced today the presentation of results from the first completed randomized clinical trial for Altrazeal at the SAWC Fall symposium in Washington D.C. September 16-18. The r... Devices, Wound CareULURU, Altrazeal (Source: HSMN NewsFeed)

The ACTH-induced cortisol response in acute pancreatitis

Critical Care — Tue, 15 Sep 2009 23:00:00 +0100

The evidence that severe acute pancreatitis can result in critical illness related corticosteroid insufficiency following impaired adrenal secretion is accumulating. The study by Peng and coworkers in Critical Care certainly contributes to that idea, even though the question whether relative adrenal insufficiency should prompt for treatment by substitution doses of corticosteroids remains unresolved. The study is discussed in terms of the risk factors, circumstances and significance of impaired corticosteroid secretion by adrenals in severe acute pancreatitis. (Source: Critical Care)

Novel and recurrent mutations in the AIRE gene of autoimmune polyendocrinopathy syndrome type 1 (APS1) patients.

Clinical Genetics — Mon, 14 Sep 2009 23:00:00 +0100

This study describes seven Arab families, in which 18 patients had APS1. In addition to the cardinal features of APS1, some patients exhibited alopecia, diabetes mellitus, nephrocalcinosis and other phenotypes associated with APS1. DNA sequencing of the AIRE gene of patients from this study identified four novel and one recurrent mutation. These mutations likely result in loss of AIRE function in the patients. In addition, it was noted that the non-pathogenic c.834C> G mutation (rs1800520, encoding for p.Ser278Arg) occurs with high incidence in the AIRE gene of Arab individuals. Furthermore, this investigation demonstrates inflammation of the hair follicles in APS1 patients with alopecia universalis. We conclude that Arab APS1 patients carry novel and recurrent mutations in the AIRE gen...

Activities against hemostatic proteins and adrenal gland ultrastructural changes caused by the brown widow spider Latrodectus geometricus (Araneae: Theridiidae) venom.

Comparative biochemistry and physiology. Toxicology and pharmacology : CBP — Fri, 11 Sep 2009 23:00:00 +0100

Authors: Guerrero B, Finol HJ, Reyes-Lugo M, Salazar AM, Sánchez EE, Estrella A, Roschman-González A, Ibarra C, Salvi I, Rodríguez-Acosta A Brown widow spider (BrWS) (Latrodectus geometricus) venom produces intense systemic reactions such as cramps, harsh muscle nociceptive, nauseas, vomiting and hypertension. The proposed pathogenic mechanisms resulting in these accidents have principally been damages occuring at the nervous system. However, it is suspected that there is also damage of the adrenal glands, as a result of the experimental animal's clinical manifestations, which developed symptoms compatible with acute adrenal insufficiency. We have currently found that the adrenal gland is damaged by this venom gland homogenates (VGH) producing severe alterations on corte...

Health notes: City

OrlandoSentinel: Medical Research — Tue, 08 Sep 2009 23:00:00 +0100

Aging program Free program for seniors 55 and older consists of social time and health topics. A guest speaker from the Cook County sheriff's office will discuss home safety. 1 p.m. Friday. Our Lady of the Resurrection Medical Center, 5645 W. Addison St.... (Source: OrlandoSentinel: Medical Research)

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John F. Kennedy's Addison's disease was probably caused by rare autoimmune disease

Los Angeles Times - Science — Sat, 05 Sep 2009 07:00:00 +0100

A Navy doctor's report sheds new light on the late president's medical records. President Kennedy's Addison's disease, which came to light only after his election in 1960, was most likely caused by a rare autoimmune disease, according to a Navy doctor who reviewed Kennedy's medical records. The disease, autoimmune polyendocrine syndrome type 2, or APS 2, also caused Kennedy's hypothyroidism, according to a report published Tuesday in the Annals of Internal Medicine . (Source: Los Angeles Times - Science)

Withdrawal of corticosteroids in inflammatory bowel disease patients after dependency periods ranging from 2 to 45 years: a proposed method

Alimentary Pharmacology and Therapeutics — Thu, 03 Sep 2009 23:00:00 +0100

Conclusions Corticosteroid withdrawal using this protocol had a high success rate and durable effect and was effective in patients with long-standing (up to 45 years) dependency. As symptoms of CWS mimic symptoms of IBD disease flares, gastroenterologists may have difficulty distinguishing them, which may be a contributory factor to the frequency of corticosteroid dependency in IBD patients. (Source: Alimentary Pharmacology and Therapeutics)

John F. Kennedy had the most complex medical history of any U.S. president, likely had polyendocrine syndrome type II

Clinical Cases and Images — Wed, 02 Sep 2009 10:37:00 +0100

From the Annals of Internal Medicine:In an Era of Less Media Scrutiny, John F. Kennedy Hid Serious Health Problems from the Public.At the age of 43, he was the youngest man ever elected president. During his campaign and presidency, the media portrayed him as the epitome of youth and vigor. However, a recent review of his medical records reveals that Kennedy had the most complex medical history of any U.S. president.Unbeknownst public, Kennedy was diagnosed with Addison's disease, a rare endocrine disorder in which the adrenal glands do not produce enough of the hormone cortisol.Later, when Kennedy was a senator, he was found to have hypothyroidism. During the 1960 campaign for the presidency, Kennedy's physician denied the Addison's diagnosis.Today, with newly available evidence, research...

John F. Kennedy's Addison's probably caused by rare autoimmune disease

Los Angeles Times - Science — Wed, 02 Sep 2009 07:00:00 +0100

A Navy doctor's report sheds new light on the late president's medical records. President Kennedy's Addison's disease, which came to light only after his election in 1960, was most likely caused by a rare autoimmune disease, according to a Navy doctor who reviewed Kennedy's medical records. Autoimmune polyendocrine syndrome type 2, or APS 2, also caused Kennedy's hypothyroidism, according to a report published Tuesday in the Annals of Internal Medicine . (Source: Los Angeles Times - Science)

Current controversies in critical illness-related corticosteroid insufficiency and glucocorticoid supplementation.

Orthopedics — Mon, 31 Aug 2009 23:00:00 +0100

Authors: Gross AK, Winstead PS The stress response, which is triggered by a number of factors, including surgery, results in activation of the hypothalamic pituitary adrenal axis and subsequent release of cortisol from the adrenal glands. Critical illness-related corticosteroid insufficiency is an inadequate corticosteroid response relative to a patient's illness; patients with critical illness-related corticosteroid insufficiency have both insufficient circulating cortisol and impaired cellular use of glucocorticoids. Corticosteroids, such as hydrocortisone, have been cited to improve survival, oxygenation, duration of mechanical ventilation, and intensive care unit-free days in critically ill patients. Perioperative glucocorticoid supplementation is also recommended in patients with ...

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Cytomegalovirus-Induced Adrenal Insufficiency in a Renal Transplant Recipient

Transplantation Proceedings — Mon, 31 Aug 2009 23:00:00 +0100

Abstract: Cytomegalovirus (CMV) is an important pathogen in organ-transplant recipients. There have been frequent reports of CMV-induced adrenal insufficiency in patients with human immunodeficiency virus infection. Herein, we report CMV-induced renal insufficiency in a renal transplant recipient. A 24-year-old woman had gradual onset of weakness, anorexia, nausea, hypotension, and skin hyperpigmentation at 5 months after renal transplantation. The immunosuppression regimen included cyclosporine, mycophenolate mofetil, and corticosteroid (prednisolone, 5 mg/d). Recent history included acute CMV infection, which was treated with ganciclovir. Basal serum cortisol concentration was 4 μg/dL, and stimulated serum cortisol concentration was less than 10 μg/dL. All clinical signs and symptoms a...

JFK's Health Problems More Complex Than Thought

MedlinePlus Health News — Mon, 31 Aug 2009 21:00:00 +0100

President suffered from a rare autoimmune condition, new report finds Source: HealthDay Related MedlinePlus Topics: Addison's Disease, Thyroid Diseases (Source: MedlinePlus Health News)

Severe sepsis in cirrhosis

Hepatology — Mon, 31 Aug 2009 00:00:00 +0100

Sepsis is physiologically viewed as a proinflammatory and procoagulant response to invading pathogens. There are three recognized stages in the inflammatory response with progressively increased risk of end-organ failure and death: sepsis, severe sepsis, and septic shock. Patients with cirrhosis are prone to develop sepsis, sepsis-induced organ failure, and death. There is evidence that in cirrhosis, sepsis is accompanied by a markedly imbalanced cytokine response ("cytokine storm"), which converts responses that are normally beneficial for fighting infections into excessive, damaging inflammation. Molecular mechanisms for this excessive proinflammatory response are poorly understood. In patients with cirrhosis and severe sepsis, high production of proinflammatory cytokines seems to play a...

The diagnosis and investigation of adrenal insufficiency in adults

Annals of Clinical Biochemistry — Sun, 30 Aug 2009 23:00:00 +0100

There is considerable variation in the methods used to diagnose and investigate adrenal insufficiency in clinical practice. These include a range of adrenocorticotropin (ACTH) stimulation and other dynamic testing protocols, serum cortisol cut-off values for diagnosis and tests used for differential diagnosis. With the introduction of modern cortisol and ACTH assays, the interpretation of tests used for diagnosis and differential diagnosis has become more complex and requires local validation. This review examines the basis of normal hypothalamic–pituitary–adrenal axis function and adrenal insufficiency states based upon an evidence base accumulated over the past four decades. The role of the laboratory in the differential diagnosis and interpretation based upon assay methodolo...

[Ascending hyperkaliemic paralysis revealing adrenal insufficiency.]

Annales Francaises d'Anesthesie et de Reanimation — Fri, 28 Aug 2009 23:00:00 +0100

Authors: Lehot JJ, Cahen R PMID: 19720495 [PubMed - as supplied by publisher] (Source: Annales Francaises d'Anesthesie et de Reanimation)

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Acute adrenal crisis mimicking familial Mediterranean fever attack in a renal transplant FMF patient with amyloid goiter

Rheumatology International — Thu, 27 Aug 2009 16:33:23 +0100

We describe a 21-year-old-FMF amyloidosis case with a well-functioning allograft who presented to the emergency clinic with the complaints of abdominal pain, vomiting and diarrhea mimicking FMF attack. adrenocorticotrophic hormone stimulation test was performed due to resistant hyponatremia and disclosed Addison disease. In countries with a high prevalence of FMF, adrenal crisis should be borne in mind in long standing FMF patients. Content Type Journal ArticleCategory Case ReportDOI 10.1007/s00296-009-1115-0Authors Hamdi Emeksiz, Gazi University Ankara TurkeySevcan Bakkaloglu, Gazi University Ankara TurkeyOrhun Camurdan, Gazi University Ankara TurkeyMehmet Boyraz, Gazi University Ankara TurkeyOguz Soylemezoglu, Gazi University Ankara TurkeyEnver Hasanoglu, Gazi University Ankara ...

Effects of glucose infusion on neuroendocrine and cognitive parameters in Addison disease

Metabolism - Clinical and Experimental — Wed, 26 Aug 2009 00:00:00 +0100

Abstract: Sucrose intake has been shown to suppress increased adrenocorticotropic hormone (ACTH) levels in adrenalectomized rats, suggesting that increased cerebral energy supply can compensate for the loss of glucocorticoid feedback inhibition of the hypothalamo-pituitary-adrenal axis. We hypothesized that glucose infusion might acutely down-regulate increased ACTH secretion in patients with Addison disease. We studied 8 patients with primary adrenal insufficiency (Addison group) with short-term discontinuation of hydrocortisone substitution and 8 matched healthy controls in 2 randomized conditions. Subjects received either intravenous glucose infusion (0.75 g glucose per kilogram body weight for 2.5 hours) or placebo. Concentrations of ACTH, cortisol, catecholamines, growth hormone, gluc...

Recent Advances in Adrenal Autoimmunity

Medscape Diabetes Headlines — Tue, 25 Aug 2009 21:36:42 +0100

Primary adrenal insufficiency (PAI), also known as Addison’s disease, is the result of the destruction or impaired function of adrenocortical cells. An autoimmune process is responsible. Expert Review of Endocrinology and Metabolism (Source: Medscape Diabetes Headlines)

Predisposing Factors for Adrenal Insufficiency

New England Journal of Medicine — Tue, 18 Aug 2009 23:00:00 +0100

To the Editor: The review of predisposing factors for adrenal insufficiency by Bornstein (May 28 issue)1 includes a welcome discussion ... (Source: New England Journal of Medicine)

Addison’s disease due to tuberculosis that required differentiation from SIADH

Journal of Infection and Chemotherapy — Mon, 17 Aug 2009 19:00:10 +0100

Abstract A 77-year-old man was admitted to our hospital complaining of general fatigue. Serum sodium was 116 mEq/l and serum antidiuretic hormone (ADH) was elevated. Radiologic examination revealed nodules in the brain as well as in both adrenal glands. Based on the findings of fluorine-18 fluorodeoxyglucose positron emission tomography (FDG-PET), we had considered that the cause of the hyponatremia was syndrome of inappropriate secretion of antidiuretic hormone (SIADH) due to active extrapulmonary tuberculosis. Against our expectations, the patient’s condition got worse just after he began antituberculous therapy; we finally diagnosed Addison’s disease by additional hormonal tests. His condition recovered immediately with the administration of high-dose hydrocortisone,...

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Levothyroxine sodium: Adrenal insufficiency: case report

Reactions — Mon, 17 Aug 2009 10:19:17 +0100

(Source: Reactions)

Vasopressin use is associated with death in acute trauma patients with shock

Journal of Critical Care — Mon, 17 Aug 2009 00:00:00 +0100

Conclusions: Arginine vasopressin is associated with increased mortality in trauma patients with refractory hypotension. Arginine vasopressin may be a marker of illness or possibly play a causal role in adverse outcomes. Clinicians should reconsider expanding the indications of AVP use. (Source: Journal of Critical Care)

Growth hormone replacement in adults: interactions with other pituitary hormone deficiencies and replacement therapies.

European Journal of Endocrinology — Thu, 13 Aug 2009 23:00:00 +0100

Authors: Filipsson H, Johannsson G Severe growth hormone deficiency (GHD) in adults has been described as a clinical entity. Some of the features associated with GHD could, however, be due to unphysiological and inadequate replacement of other pituitary hormone deficiencies. This may be true for glucocorticoid replacement that lacks a biomarker making dose titration and monitoring difficult. Moreover, oral oestrogen replacement therapy decreases insulin growth factor 1 (IGF-I) levels compared to transdermal route, which attenuates the responsiveness to GH replacement therapy in women. In addition, in untreated female hypogonadism, oral oestrogen may augment the features associated with GHD in adult women. Important interactions between the hormones used for replacing pituitary hormone ...

[Adrenal function after induction of cardiac surgery patients with etomidate: A retrospective study.]

Annales Francaises d'Anesthesie et de Reanimation — Thu, 13 Aug 2009 23:00:00 +0100

CONCLUSION: A high incidence of relative adrenal failure was observed in selected cardiac surgery patients with acute postoperative circulatory failure. PMID: 19683891 [PubMed - as supplied by publisher] (Source: Annales Francaises d'Anesthesie et de Reanimation)

Researching nature's venoms and poisons

Transactions of the Royal Society of Tropical Medicine and Hygiene — Thu, 13 Aug 2009 12:40:54 +0100

Summary: Our environment hosts a vast diversity of venomous and poisonous animals and plants. Clinical toxinology is devoted to understanding, preventing and treating their effects in humans and domestic animals. In Sri Lanka, yellow oleander (Thevetia peruviana, Sinhala ‘kaneru’), a widespread and accessible ornamental shrub, is a popular means of self-harm. Its toxic glycosides resemble those of foxglove, against which therapeutic antibodies have been raised. A randomised placebo-controlled trial proved that this treatment effectively reversed kaneru cardiotoxicity. There are strong scientific grounds for the use of activated charcoal, but encouraging results with multiple-dose activated charcoal were not confirmed by a recent more powerful study. Venom of Russell's viper (Daboia sia...

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The Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy or Autoimmune Polyglandular Syndrome Type 1

Seminars in Liver Disease — Thu, 13 Aug 2009 10:53:53 +0100

Semin Liver Dis 2009; 29: 307-314DOI: 10.1055/s-0029-1233535ABSTRACTAutoimmune polyglandular syndromes are rare autoimmune endocrinopathies that are associated with nonendocrine autoimmunopathies. Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED), also named autoimmune polyglandular syndrome type 1 (APS-1), is distinguished from autoimmune polyglandular syndrome 2 (APS-2). Major disease components of APECED are adrenal insufficiency, hypoparathyroidism, and candidiasis. The diagnosis is established by the presence of two out of the three components. Minor clinical features include autoimmune hepatitis, which occurs in up to 20% of APECED patients, and ranges from a mild to a fulminant course. The disease mostly affects juvenile patients from Sardegna, Italy, Finland, ...

Surgery in the patient with endocrine dysfunction.

The Medical Clinics of North America — Thu, 13 Aug 2009 10:24:21 +0100

A novel mutation in DAX1 (NR0B1) causing X-linked adrenal hypoplasia congenita: clinical, hormonal and genetic analysis

Endocrine — Tue, 11 Aug 2009 17:06:19 +0100

We describe a family with three members affected, two adults and a neonate. The way of presentation of the adults was neonatal primary adrenal insufficiency and hypogonadotropic hypogonadism (one complete and another presenting as interrupted puberty). The genetic study revealed a new mutation in DAX1, p.Q76X gene (c.C226T), resulting in a truncated protein of 76 amino acids, the same in all three affected male patients and in the asymptomatic women of the family. These cases further expand the number of DAX1 mutations reported, as well as the description of infrequent forms of presentation of this disease as interrupted puberty. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s12020-009-9232-9Authors Katherine García-Malpartida, Doctor Peset University Hospital E...

Society produces swine flu information sheet for patients taking hydrocortisone

Society for Endocrinology — Tue, 11 Aug 2009 12:00:00 +0100

The Society for Endocrinology Clinical Committee in association with the Addison’s Disease Self Help Group and the Pituitary Foundation have prepared a patient information sheet advising patients taking hydrocortisone on how to prepare for swine flu. The information sheet and accompanying press release can be downloaded from the SfE homepage or by clicking on the link below.Swine flu information sheetPress release (Source: Society for Endocrinology)

Surgery in the Patient with Endocrine Dysfunction

Medical Clinics of North America — Sat, 08 Aug 2009 11:29:34 +0100

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[Associations of autoimmune disorders in endocrine diseases.]

Orvosi Hetilap — Thu, 06 Aug 2009 00:38:23 +0100

Authors: Balázs C, Fehér J Increasing data are known for dialogue between neuroendocrine and immune systems recently. Results of molecular genetic studies provided evidences for common languages of these systems by various signals including neurotransmitters, hormones, cytokines. It is proved the immune system is able to produce neurotransmitters and hormones and endocrine organs can even result in cytokines. This new integrative approach allows to investigate the physiologic events and diseases as interactions between the psycho-neuro-endocrine-immune systems. The autoimmune polyendocrine syndromes constitute a heterogeneous group of disorders characterized by loss of immune tolerance to self-antigens. In spite of distinct clinical pictures, molecular genetic studies reveale...

A novel copper(II) complex of a tripodal ligand with phenolate-phenol interligand, intramolecular hydrogen bonding

RSC - Dalton Trans. latest articles — Wed, 05 Aug 2009 23:00:00 +0100

Vaidyanathan Mathrubootham, Anthony W. Addison, K. Travis Holman, Ekkehard Sinn, Laurence K. Thompson (Paper from Dalton Trans.) Vaidyanathan Mathrubootham, Dalton Trans., 2009, DOI: 10.1039/b910188h To cite this article before page numbers are assigned, use the DOI form of citation above. The content of this RSS Feed (c) The Royal Society of Chemistry (Source: RSC - Dalton Trans. latest articles)

Antifungals/calcium channel antagonists/inhaled corticosteroids/protease inhibitors interaction: Cushing's syndrome and adrenal insufficiency: 15 case reports

Reactions — Mon, 03 Aug 2009 10:55:12 +0100

(Source: Reactions)

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

J Korean Med Sci — Fri, 31 Jul 2009 23:00:00 +0100

This study was undertaken to evaluate the incidence and risk factors associated with relative adrenal insufficiency (RAI) in Korean critically-ill patients. All patients who were admitted to the Medical Intensive Care Unit (MICU) of Samsung Medical Center between January 1, 2006 and April 30, 2007 were prospectively evaluated using a short corticotropin stimulation test on the day of admission. RAI was defined as an increase in the serum cortisol level of <9 microg/dL from the baseline after administration of 250 microg of corticotropin. In all, 123 patients were recruited and overall the incidence of RAI was 44% (54/123). The presence of septic shock (P=0.001), the Simplified Acute Physiology Score (SAPS) II (P=0.003), the Sequential Organ Failure Assessment (SOFA) score (P=0.001), the...

Addison's disease

Medicine — Fri, 31 Jul 2009 11:53:27 +0100

Abstract: Addison's disease or primary adrenocortical failure is a rare condition, most commonly caused in the UK by autoimmune destruction of the adrenal glands. The insidious onset of symptoms over many months means there is often a delay in diagnosis and patients can first present in adrenal crisis. The diagnosis is made by measurement of a low serum cortisol at 9 a.m. in the presence of an elevated adrenocorticotrophic hormone (ACTH) or by a poor cortisol response to exogenous ACTH on provocation testing. There are many possible causes of adrenal failure and once the diagnosis of adrenal insufficiency has been made these should be differentiated further. Replacement with hydrocortisone and fludrocortisone should approximate physiological levels as closely as possible and be monitored c...

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Prevalence of anti-thyroid peroxidase and anti-adrenal 21-hidroxylase in type 1 diabetes patients

Arquivos Brasileiros de Endocrinologia e Metabologia — Fri, 31 Jul 2009 10:54:51 +0100

CONCLUSIONS: Although the frequency of anti-TPO in this sample was similar to previous studies, a higher prevalence of anti-21-OH was found. However, no coexisting adrenal dysfunction was detected, which does not support universal screening for AD in this group. (Source: Arquivos Brasileiros de Endocrinologia e Metabologia)

International Perspectives: Late-onset Circulatory Collapse in Very Low-birthweight Infants: A Japanese Perspective

NeoReviews recent issues — Thu, 30 Jul 2009 23:00:00 +0100

The number of reports of sudden deterioration in blood pressure with concomitant decrease of urine output among extremely low-birthweight (ELBW) infants whose general condition is stable have increased recently in Japan. This disorder is clearly different from an unstable circulatory status during the acute phase soon after delivery. The disorder usually occurs more than 1 week after birth, when the infant's circulatory status already is stable. Blood pressure in such hypotensive infants cannot be restored with volume expanders or inotropic agents; it only improves with glucocorticoid administration. The efficacy of steroids in preterm infants who develop hypotension soon after birth is widely known, but the time of onset of this paroxysmal hypotension is apparently different. Relative adr...

Anti-CTLA-4 antibody therapy associated autoimmune hypophysitis: serious immune related adverse events across a spectrum of cancer subtypes

Pituitary — Tue, 28 Jul 2009 18:23:31 +0100

We describe the first two cases of hypopituitarism in prostate cancer subjects undergoing experimental therapy with ipilimumab. The clinical evidence strongly suggests that the prostate cancer subjects developed autoimmune hypophysitis as a consequence of anti-CTLA-4 treatment. High dose glucocorticoid treatment resulted in markedly improved symptoms, and resolution of focal symptoms and diabetes insipidus. One subject recovered pituitary-thyroid axis function after 9 months; however, both continue to require GC replacement. These cases highlight the importance of early screening and treatment for hypopituitarism in all subjects undergoing treatment with anti-CTLA-4 therapy to prevent a potentially fatal outcome from secondary adrenal insufficiency, a readily treatable disease....

Revisiting the validity of APACHE II in the trauma ICU: Improved risk stratification in critically injured adults

Injury — Tue, 28 Jul 2009 11:27:34 +0100

Conclusion: Critically injured patients have physiologic derangements not accurately accounted for by commonly used trauma scores. In this subset a more general ICU scoring system is useful for risk adjustment for research, administrative and quality improvement purposes. (Source: Injury)