MedWorm: Adrenal Insufficiency

Cardiac dysfunction in cirrhosis – does adrenal function play a role? A hypothesis

Liver International — Tue, 31 Jan 2012 05:00:00 +0100

AbstractCirrhotic cardiomyopathy (CCM), a condition of unknown pathogenesis, is characterized by suboptimal ventricular contractile response to stress, diastolic dysfunction and QT interval prolongation. It is most often found in patients with advanced cirrhosis. It is clinically relevant during stressful conditions, such as sepsis, bleeding and surgery. CCM reverses after liver transplantation and potentially has a role in the pathogenesis of hepatorenal syndrome. In adrenal insufficiency (AI), cardiac dysfunction is a feature with low ejection fraction, decreased left ventricular chamber size and electrocardiographic abnormalities, including QT interval prolongation. With optimal diagnostic tests, AI is present in approximately 10% of patients with cirrhosis, particularly in those with a...

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Adrenal insufficiency in children with sepsis

Revista Brasileira de Terapia Intensiva — Fri, 27 Jan 2012 15:57:11 +0100

CONCLUSION: This study determined the frequency of adrenal insufficiency in children with sepsis and its relationship to increased mortality within the first 28 post-admission days. No statistically significant association was found between adrenal insufficiency and mechanical ventilation time or the use of vasoactive drugs. (Source: Revista Brasileira de Terapia Intensiva)

Fludrocortisone/prednisolone/psyllium interaction: Adrenal insufficiency crisis (first report for fludrocortisone and psyllium): case report

Reactions — Mon, 23 Jan 2012 18:35:21 +0100

(Source: Reactions)

Exogenous adrenal insufficiency

About.com Eating Disorders — Fri, 20 Jan 2012 05:00:00 +0100

is a condition of low levels of hormones released by the adrenal glands, caused by factors other than problems with the glands themselves. See for information on adrenal deficiency caused by problems within these glands. (Source: About.com Eating Disorders)

Nocturnal Hypoglycemia Identified by a Continuous Glucose Monitoring System in Patients with Primary Adrenal Insufficiency (Addison's Disease)

Diabetes Technology — Thu, 19 Jan 2012 04:04:40 +0100

Diabetes Technology & Therapeutics , Vol. 0, No. 0. (Source: Diabetes Technology)

Adrenal Insufficiency as a Possible Contributor to Pain in Adults With Sickle Cell Disease (SCD) (413-B)

Journal of Pain and Symptom Management — Thu, 19 Jan 2012 03:24:50 +0100

Recognize the relationship between adrenal insufficiency and pain. (Source: Journal of Pain and Symptom Management)

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Diagnostic Value of Salivary Cortisol in the CRH Stimulation Test in Premature Infants.

The Journal of Clinical Endocrinology and Metabolism — Wed, 18 Jan 2012 05:00:00 +0100

Conclusion:This study indicated that using salivary cortisol measurements for the CRH test could be a reliable method for evaluating the hypothalamic-pituitary-adrenal axis in VLBWI with gestational age of less than 29 wk. PMID: 22259060 [PubMed - as supplied by publisher] (Source: The Journal of Clinical Endocrinology and Metabolism)

Vanishing white matter disease caused by EIF2B2 mutation with the presentation of an adrenoleukodystrophy phenotype.

Gene — Tue, 17 Jan 2012 05:00:00 +0100

This report widens the clinical spectrum of VWMD and raises the possibility of an allele-specific association with adrenal insufficiency. PMID: 22285377 [PubMed - as supplied by publisher] (Source: Gene)

Lipodystrophy and adrenal insufficiency: Potential mediators of peripheral neuropathy in HIV infection?

Medical Hypotheses — Mon, 16 Jan 2012 05:00:00 +0100

Abstract: The mechanisms behind certain co-morbid conditions associated with chronic HIV disease still remain elusive. HIV-associated peripheral neuropathy is one among those rarely studied manifestations in HIV-1 infection. Numerous underlying factors associated with peripheral neuropathy have been described in HIV disease. Herein, we hypothesized certain heretofore undescribed potential mechanisms that lead to HIV associated neuropathy. Being a multifactoral manifestation, HIV-associated neuropathy is presumed to have an association with physiological factors namely, adrenal inadequacy/steroid resistance and lipodystrophy-induced cushion-effect loss in peripheral nerves. Therefore, management of the adrenals with steroids at the time-point of high inflammatory burden thereby preventing l...

Nocturnal Hypoglycemia Identified by a Continuous Glucose Monitoring System in Patients with Primary Adrenal Insufficiency (Addison's Disease)

Diabetes Technology — Sat, 14 Jan 2012 04:05:09 +0100

Diabetes Technology & Therapeutics , Vol. 0, No. 0. (Source: Diabetes Technology)

Busulfan/cyclophosphamide: Adrenal insufficiency in a child: case report

Reactions — Fri, 13 Jan 2012 23:22:43 +0100

(Source: Reactions)

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Adrenocortical dysfunction in liver disease: A systematic review

Hepatology — Wed, 11 Jan 2012 05:00:00 +0100

AbstractIn patients with cirrhosis adrenal insufficiency (AI) is reported during sepsis and septic shock, and is associated with increased mortality. Consequently, the term “hepato‐adrenal syndrome” was proposed. Some studies have shown that AI is frequent in stable cirrhosis, and in cirrhosis associated with decompensation other than sepsis, such as bleeding and ascites. Moreover, other studies showed a high prevalence in liver transplant recipients, immediately after or some time after liver transplantation. The effect of corticosteroid therapy in critically ill patients with liver disease has been evaluated in some studies, but the results remain controversial.The 250 µg ACTH stimulation test to diagnose AI in critically ill adult patients is recommended by an international task ...

Long-term Management for Primary Adrenal InsufficiencyLong-term Management for Primary Adrenal Insufficiency

Medscape Today Headlines — Wed, 04 Jan 2012 04:00:00 +0100

Learn about glucocorticoid replacement for special situations such as pregnancy and hypertension. Clinical Endocrinology (Source: Medscape Today Headlines)

Salivary Cortisol as a Diagnostic Tool for Cushing's Syndrome and Adrenal Insufficiency: Improved Screening by an Automatic Immunoassay.

European Journal of Endocrinology — Tue, 03 Jan 2012 05:00:00 +0100

Conclusions: The newly established thresholds facilitated initial screening for secondary adrenal insufficiency and allowed excellent identification of hypercortisolism. Measurement by an automated immunoassay will allow broader use of salivary cortisol as a diagnostic tool. PMID: 22214924 [PubMed - as supplied by publisher] (Source: European Journal of Endocrinology)

Discordant growth hormone and IGF-1 levels post pituitary surgery in patients with acromegaly naïve to medical therapy and radiation: what to follow, GH or IGF-1 values?

Pituitary — Mon, 19 Dec 2011 16:51:07 +0100

Abstract New criteria that define acromegaly remission are more stringent: normal (age/sex-adjusted) insulin-like growth factor type 1 (IGF-1), growth hormone (GH) random (GHr) <1 μg/L, and a GH nadir (GHn) during oral glucose tolerance test (OGTT) of <0.4 μg/L. Discordance between GH and IGF-1 values is often attributed to somatostatin receptor ligands (SRLs) or radiation. The purpose of this study was to evaluate rates of discordant IGF-1 and GH levels in patients with GH secreting adenomas (after pituitary surgery), who were naïve to any other treatment. We retrospectively analyzed data over a 5 year time period (2006–2010), in post-surgery acromegaly patients who had elevated IGF-1 but normal GH levels (per the new cure criteria). Symptoms of a...

Suppression of the HPA axis during extrahepatic biliary obstruction induces cholangiocyte proliferation in the rat

AJP: Gastrointestinal and Liver Physiology — Mon, 19 Dec 2011 05:00:00 +0100

Cholestatic patients often present with clinical features suggestive of adrenal insufficiency. In the bile duct-ligated (BDL) model of cholestasis, the hypothalamic-pituitary-adrenal (HPA) axis is suppressed. The consequences of this suppression on cholangiocyte proliferation are unknown. We evaluated 1) HPA axis activity in various rat models of cholestasis and 2) effects of HPA axis modulation on cholangiocyte proliferation. Expression of regulatory molecules of the HPA axis was determined after BDL, partial BDL, and α-naphthylisothiocyanate (ANIT) intoxication. The HPA axis was suppressed by inhibition of hypothalamic corticotropin-releasing hormone (CRH) expression by central administration of CRH-specific Vivo-morpholinos or by adrenalectomy. After BDL, the HPA axis was reactiva...

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What Causes a Black Colored Tongue?

PediatricEducation.org — Mon, 19 Dec 2011 00:29:33 +0100

Discussion Although dental caries, strep throat and oral candidiasis are some of the most common oral pathology. The tongue itself can be a source of potential pathology. Geographic tongue, oral candidiasis, and lingual ulcerations are common problems. Most discolorations of the tongue are because of food, drink or medications that are ingested and are self-limited. However discoloration can be a sign of more significant problems. Hairy tongue, often colored black, is a relatively uncommon problem in the US but has a higher incidence reported in Turkey and Iran. It is more common in adults than children but has been reported in a child 2 months of age. It is also called lingua villosa nigra and is a benign condition caused by keratin accumulation usually in the setting of poor oral hygiene...

Successful management of cryptococcosis of the bilateral adrenal glands and liver by unilateral adrenalectomy with antifungal agents: a case report

BMC Infectious Diseases — Wed, 14 Dec 2011 05:00:00 +0100

Conclusions: This is the first report describing adrenal cryptococcosis with adrenal insufficiency and liver invasion without central nervous system involvement. Adrenal cryptococcosis should be considered in the differential diagnosis for patients with bilateral adrenal masses with primary adrenal deficiency. Unilateral adrenalectomy was quite effective in controlling the cryptococcosis in this case. Even in patients with bilateral adrenal cryptococcosis, unilateral adrenalectomy should be an option for treatment of disseminated cryptococcosis. (Source: BMC Infectious Diseases)

[131I]Iodometomidate for Targeted Radionuclide Therapy of Advanced Adrenocortical Carcinoma.

The Journal of Clinical Endocrinology and Metabolism — Wed, 14 Dec 2011 05:00:00 +0100

Conclusions:Radionuclide therapy with [(131)I]IMTO is a promising treatment option for selected patients with ACC, deserving evaluation in prospective clinical trials. PMID: 22170726 [PubMed - as supplied by publisher] (Source: The Journal of Clinical Endocrinology and Metabolism)

What is the best long‐term management strategy for patients with primary adrenal insufficiency?

Clinical Endocrinology — Wed, 07 Dec 2011 22:06:23 +0100

This article provides answers to the important daily clinical questions, such as correct dose finding, dose adaptation in special situations, e g, pregnancy, improvement of quality of life and measures for protection from adrenal crisis. Other important aspects discussed are side effects of glucocortiocid replacement therapy and interactions with other drugs. (Source: Clinical Endocrinology)

Genotype-Phenotype Analysis in Congenital Adrenal Hyperplasia due to P450 Oxidoreductase Deficiency.

The Journal of Clinical Endocrinology and Metabolism — Wed, 07 Dec 2011 05:00:00 +0100

Conclusions:We report clinical, biochemical, and genetic findings in a large PORD cohort and show that MLPA is a useful addition to POR mutation analysis. Homozygosity for the most frequent mutation in Caucasians, p.A287P, allows for prediction of genital phenotype and moderate malformations. Adrenal insufficiency is frequent, easily overlooked, but readily detected by cosyntropin testing. PMID: 22162478 [PubMed - as supplied by publisher] (Source: The Journal of Clinical Endocrinology and Metabolism)

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Serum and salivary cortisol in the diagnosis of adrenal insufficiency and as a predictor of the outcome in patients with severe sepsis

Arquivos Brasileiros de Endocrinologia e Metabologia — Sun, 04 Dec 2011 08:16:21 +0100

CONCLUSIONS: Salivary cortisol in critically ill patients may be a better laboratory indicator of cortisol levels than serum total cortisol.OBJETIVOS: Comparar cortisol salivar com sérico total em pacientes com sepse grave, em pós-operatório e controles normais. MATERIAIS E MÉTODOS: Cortisol sérico total foi determinado por imunoensaio quimioluminescente e cortisol salivar por imunoensaio enzimático. RESULTADOS: Em pacientes com sepse grave, a mediana do cortisol salivar foi 14,0 e 2,6 vezes maior que dos pacientes em pós-operatório e saudáveis. Nos pacientes em pós-operatório, cortisol salivar foi 5,4 vezes maior que o controle. Cortisol sérico total também foi maior em pacientes com sepse grave que nos saudáveis e pós-operatórios, porém, esse incremento foi bem menor (2,...

Adrenal disease in pregnancy

Best Practice and Research. Clinical Endocrinology and Metabolism — Fri, 25 Nov 2011 08:02:59 +0100

Adrenal disorders in pregnancy are relatively rare, yet can lead to significant maternal and fetal morbidity. Making a diagnosis is challenging as pregnancy may alter the manifestation of disease, many signs and symptoms associated with pregnancy are also seen in adrenal disease, and the fetal-placental unit alters the maternal endocrine metabolism and hormonal feedback mechanisms. The most common cause of Cushing’s syndrome in pregnancy is an adrenal adenoma, followed by pituitary etiology, adrenal carcinoma, and other exceedingly rare causes. Medical therapy of Cushing’s syndrome includes metyrapone and ketoconazole, but generally surgical treatment is more effective. Exogenous corticosteroid administration is the most common cause of adrenal insufficiency, followed by the endogenous...

SfE awards second Public Engagement Grant

Society for Endocrinology — Thu, 24 Nov 2011 12:00:00 +0100

Dr Niki Karavitaki, Consultant Endocrinologist, Oxford, will deliver two ?Steroid replacement study days’ to educate patients with adrenal insufficiency and their family members/carers about their condition and medication. These events are sponsored by SfE through a Public Engagement Grant awarded to Dr Karavitaki. The study days will take place at the Oxford Centre for Diabetes, Endocrinology & Metabolism. Dr Karavitaki will be joined by Specialty Registrars and an Endocrine Nurse team to deliver educational talks and a practical demonstration of the emergency steroid jabs. Previous study days have received excellent feedback and have improved attendees’ confidence in giving the emergency steroid jab. The events will take place from 17:00-18:30 on Monday 5 March 2012, and...

Improved Cortisol Exposure-Time Profile and Outcome in Patients with Adrenal Insufficiency: A Prospective Randomized Trial of a Novel Hydrocortisone Dual-Release Formulation.

The Journal of Clinical Endocrinology and Metabolism — Wed, 23 Nov 2011 05:00:00 +0100

Conclusion:The OD dual-release tablet provided a more circadian-based serum cortisol profile. Reduced body weight, reduced blood pressure, and improved glucose metabolism were observed during OD treatment. In particular, glucose metabolism improved in patients with concomitant DM. PMID: 22112807 [PubMed - as supplied by publisher] (Source: The Journal of Clinical Endocrinology and Metabolism)

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Hyponatremia and antidiuresis syndrome.

Annales d'Endocrinologie — Wed, 23 Nov 2011 05:00:00 +0100

Authors: Vantyghem MC, Balavoine AS, Wémeau JL, Douillard C Abstract Antidiuretic hormone (ADH), or arginine vasopressin (AVP), is primarily regulated through plasma osmolarity, as well as non-osmotic stimuli including blood volume and stress. Links between water-electrolyte and carbohydrate metabolism have also been recently demonstrated. AVP acts via the intermediary of three types of receptors: V1a, or V1, which exerts vasoconstrictive effects; pituitary gland V1b, or V3, which participates in the secretion of ACTH; and renal V2, which reduces the excretion of pure water by combining with water channels (aquaporin 2). Antidiuresis syndrome is a form of euvolaemic, hypoosmolar hyponatraemia, which is characterised by a negative free water clearance with inappropriate urine osmol...

Adrenal Failure due to Adrenal Metastasis of Lung Cancer: A Case Report

Infectious Diseases in Obstetrics and Gynecology — Tue, 22 Nov 2011 15:56:52 +0100

We report a case of a patient with adrenal failure due to bilateral adrenal metastasis of lung cancer. This is a rare presentation of lung cancer. We review the differential diagnosis of weight loss and how to make diagnosis of adrenal insufficiency. (Source: Infectious Diseases in Obstetrics and Gynecology)

Cardiovascular instability after patent ductus arteriosus ligation in preterm infants: the role of hydrocortisone

Journal of Perinatology — Thu, 17 Nov 2011 05:00:00 +0100

Authors: M D Satpute, P K Donohue, L Vricella & S W Aucott (Source: Journal of Perinatology)

Limited contribution of NR5A1 (SF-1) mutations in women with primary ovarian insufficiency (POI)

Fertility and Sterility — Thu, 17 Nov 2011 05:00:00 +0100

Conclusion(s): The current study demonstrates that mutations in the NR5A1 gene are rare in women with POI. Primary ovarian insufficiency remains unexplained in the great majority of patients; therefore, continued efforts are needed to elucidate its underlying genetic factors. (Source: Fertility and Sterility)

Congenital lipoid adrenal hyperplasia (a rare form of adrenal insufficiency and ambiguous genitalia) caused by a novel mutation of the steroidogenic acute regulatory protein gene

European Journal of Pediatrics — Mon, 14 Nov 2011 16:53:27 +0100

Conclusion: There is significant phenotypic variability among patients with lipoid CAH. While splice site mutations in the StAR gene lead to premature translational termination, resulting in truncated and non-functional proteins, there is phenotypic variability among patients with such mutations. Our patient appears to have the more atypical phenotype compared to reported patients with similar mutations. The molecular mechanism underlying this heterogeneity remains unclear. Content Type Journal ArticleCategory Original PaperPages 1-7DOI 10.1007/s00431-011-1620-5Authors Oksana Lekarev, Department of Pediatrics, Mount Sinai School of Medicine, Box 1198, One Gustave L. Levy Place, New York, NY 10029, USADelphine Mallet, Molecular Endocrinology and Rare Diseases, Hospices Civils de Ly...

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DuoCort Plenadren drug receives European approval

Pharmaceutical Technology — Thu, 10 Nov 2011 19:36:23 +0100

The European Commission has granted a European Marketing Authorisation for DuoCort Pharma's Plenadren, an orphan drug used to treat adrenal insufficiency in adults.Post to:Delicious Digg reddit Facebook StumbleUpon (Source: Pharmaceutical Technology)

Atazanavir/ritonavir/triamcinolone interaction: Cushing syndrome with secondary adrenal insufficiency: case report

Reactions — Thu, 10 Nov 2011 08:03:53 +0100

(Source: Reactions)

Plenadren® (modified release hydrocortisone tablet) approved in the EU for adrenal insufficiency

NeLM - News — Tue, 08 Nov 2011 05:00:00 +0100

Source: PharmaLive Area: News According to a PharmaLive report, the European Commission has granted a European Marketing Authorisation for Plenadren® (hydrocortisone, modified release tablet), an orphan drug for treatment of adrenal insufficiency in adults. This product is a dual release tablet given once daily - an outer layer releases hydrocortisone immediately and an inner core releases the rest of the drug more slowly during the day. It has been designed to better mimic the normal physiological cortisol profile, in order to improve outcomes for patients suffering from adrenal insufficiency. (Source: NeLM - News)

Is there a real adrenal axis dysfunction in patients with amyloidosis associated with familial Mediterranean fever?

Rheumatology International — Sat, 05 Nov 2011 17:07:51 +0100

Abstract Systemic amyloidosis with AA-type amyloid deposition is the major complication of FMF, leading to end stage renal disease. There is no clear data on the prevalence of adrenal involvement in patients with FMF amyloidosis. The aim of this study is to determine the adrenal axis function in patients FMF with amyloidosis. Twenty patients with FMF with amyloidosis (F/M: 10/10, mean age; 38 ± 11 SD years), twenty without amyloidosis (F/M: 14/6, mean age 32 ± 10 years), and healthy controls (F/M: 12/8, mean age: 30 ± 7.6 SD years) were recruited. A dose of 250 mg tetracosactide (Synacthen) was then administered intravenously and further blood samples collected 30 and 60 min later. Blood samples were separated and collected at...

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Glucocorticoid-induced diabetes and adrenal suppression: How to detect and manage them

Cleveland Clinic Journal of Medicine — Wed, 02 Nov 2011 04:00:00 +0100

Glucocorticoids, commonly used to treat multiple inflammatory processes, can cause hyperglycemia, Cushing syndrome, adrenal suppression, and, when they are discontinued, adrenal insufficiency. Physicians must be aware of these adverse effects and be equipped to manage them. (Source: Cleveland Clinic Journal of Medicine)

[Diagnosis of congenital endocrinological disease in newborns with prolonged jaundice and hypoglycaemia.]

Anales de Pediatria — Tue, 18 Oct 2011 04:00:00 +0100

CONCLUSIONS: Prolonged or cholestatic jaundice associated with neonatal hypoglycaemia is highly likely to be due to pituitary hormone deficiency or primary adrenal insufficiency. Early diagnosis and treatment of these children reverts the prolonged jaundice and prevents morbidity and mortality due to recurrent hypoglycaemia and hormone deficiencies. PMID: 22015010 [PubMed - as supplied by publisher] (Source: Anales de Pediatria)

Bone Mineral Density Is Not Significantly Reduced in Adult Patients on Low-Dose Glucocorticoid Replacement Therapy.

The Journal of Clinical Endocrinology and Metabolism — Wed, 12 Oct 2011 04:00:00 +0100

Conclusions:Adult PAI and CAH patients on low glucocorticoid doses showed normal BMD within the normal reference range. The use of longer acting prednisolone resulted in significantly lower BMD in PAI. In addition, DHEA treatment may have a beneficial effect on bone in Addison's women. PMID: 21994966 [PubMed - as supplied by publisher] (Source: The Journal of Clinical Endocrinology and Metabolism)

Adrenal function in Smith–Lemli–Opitz syndrome

American Journal of Medical Genetics Part A — Tue, 11 Oct 2011 04:00:00 +0100

This article is a U.S. Government work and is in the public domain in the USA. (Source: American Journal of Medical Genetics Part A)

Suppression of the HPA axis during extrahepatic biliary obstruction induces cholangiocyte proliferation in the rat.

American Journal of Physiology. Gastrointestinal and Liver Physiology — Thu, 06 Oct 2011 04:00:00 +0100

CONCLUSION: Suppression of the HPA axis is an early event following BDL and induces cholangiocyte proliferation. Knowledge of the role of the HPA axis during cholestasis may lead to the development of innovative treatment paradigms for chronic liver disease. PMID: 21979757 [PubMed - as supplied by publisher] (Source: American Journal of Physiology. Gastrointestinal and Liver Physiology)

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Fluconazole/fluticasone propionate interaction: First report of an interaction, leading to adrenal insufficiency and Cushing syndrome in a child: case report

Reactions — Mon, 03 Oct 2011 06:00:13 +0100

(Source: Reactions)

Tramadol: First report of adrenal insufficiency: case report

Reactions — Mon, 03 Oct 2011 06:00:13 +0100

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Reply

Clinical Gastroenterology and Hepatology — Mon, 03 Oct 2011 04:00:00 +0100

We report no significant difference in prevalence of AI among variceal bleeders with serum albumin levels below or above 2.5 g/dl. (Source: Clinical Gastroenterology and Hepatology)

Incidence of Adrenal Insufficiency and Impact of Corticosteroid Supplementation in Critically Ill Children with Systemic Inflammatory Syndrome and Vasopressor-Dependent Shock: Hebbar KB, Stockwell JA, Leong T, Fortenberry JD. Crit Care Med 2011;39:1145–50.

The Journal of Emergency Medicine — Sat, 01 Oct 2011 04:00:00 +0100

This study was a retrospective review of 78 patients with a median age of 84 months, who were treated at the Atlanta Pediatric Intensive Care Unit (PICU) with a protocol for steroid supplementation in patients meeting SIRS criteria. The primary outcome was decrease in vasopressor requirements after initiation of steroid treatment. All PICU patients requiring intravenous fluids and vasopressors for treatment of shock were tested for adrenal insufficiency with a standardized corticotrophin stimulation test. Patients were then classified as having absolute adrenal insufficiency (AAI) if a basal cortisol level < 18 mg/dL was measured vs. relative adrenal insufficiency (RAI) if the corticotrophin stimulation test induced a change in measured cortisol levels. Of the initial 78 patients, 44 (56%)...

The Use of Oral Budesonide in Adolescents and Adults With Protein-Losing Enteropathy After the Fontan Operation [ORIGINAL ARTICLES: PEDIATRIC CARDIAC]

The Annals of Thoracic Surgery — Wed, 28 Sep 2011 04:00:00 +0100

Conclusions CR-budesonide can be used to treat PLE in certain patients, but careful assessment of hepatic function should be performed before initiation of therapy as systemic side effects can limit treatment. Normal serum liver function tests do not preclude hepatic dysfunction in the Fontan patient, and it is important to perform radiographic assessments as well. (Source: The Annals of Thoracic Surgery)

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Low‐dose hydrocortisone treatment for patients with septic shock: A pilot study comparing 3 days with 7 days

Respirology — Mon, 26 Sep 2011 14:32:00 +0100

Conclusions: This pilot study showed that in patients with septic shock and relative adrenal insufficiency, 28‐day mortality did not differ between those treated with low‐dose hydrocortisone for 3 or 7 days. (Source: Respirology)

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

Best Practice and Research. Clinical Endocrinology and Metabolism — Thu, 22 Sep 2011 09:06:57 +0100

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

Prednisolone: Adrenal insufficiency in paediatric patients: 4 case reports

Reactions — Thu, 22 Sep 2011 07:55:36 +0100

(Source: Reactions)

Severe intestinal pseudo-obstruction following withdrawal from over-the-counter steroid abuse

Journal of Postgraduate Medicine — Thu, 22 Sep 2011 04:00:00 +0100

We report a case of a 59-year-old man who presented with diverticular bleeding and developed prolonged ileus postoperatively. After observing arthritic joints on examination, further questioning revealed long-term, high-dose steroid use for analgesic effect. Failure to produce an effective cortisol response was due to adrenal suppression from continuous steroid use. Immediate improvement of his ileus was seen after steroid replacement. Unreported self-medication is a frequent problem encountered in developing countries. RAI can be easily missed and requires a high index of suspicion in any patient who fails to respond to conventional treatment or with long-term steroid use. (Source: Journal of Postgraduate Medicine)

Iatrogenic Cushing syndrome after a single intramuscular corticosteroid injection and concomitant protease inhibitor therapy

Journal of the American Academy of Dermatology — Sun, 18 Sep 2011 01:35:52 +0100

We describe a 41-year-old HIV-positive woman on a regimen of lamivudine, tenofovir, atazanavir, and ritonavir who developed a diffuse, pruritic, nonspecific dermatitis that was unresponsive to medium-potency topical corticosteroids who was treated with a single intramuscular (IM) injection of 60 mg triamcinolone acetonide (TAC). After missing her follow-up appointment, the patient presented at 3 months with resolution of her eruption, weight gain of 7 kg, round facies, and increased density of coarse hairs on the face. She had lower than baseline blood pressure of 92/50 mm Hg, adrenocorticotropic hormone level less than 5 pg/mL (normal 7-50 pg/mL), and endogenous cortisol level 2.2 μg/dL (normal 5-23 μg/mL), with normal testosterone, dehydroepiandrosterone, and thyroid-stimulating hormon...

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Haemodynamic consequences of etomidate administration in elective cardiac surgery: a randomized double-blinded study

British Journal of Anaesthesia — Thu, 08 Sep 2011 04:00:00 +0100

Conclusions A single bolus of etomidate blunts the hypothalamic–pituitary–adrenal axis response for more than 24 h in patients undergoing elective cardiac surgery, but this was not associated with an increase in vasopressor requirements. (Source: British Journal of Anaesthesia)

Challenges in Management of Primary Hypoparathyroidism Associated with Autoimmune Polyglandular Syndrome Type 1

Infectious Diseases in Obstetrics and Gynecology — Wed, 07 Sep 2011 23:58:45 +0100

We report a case of autoimmune polyglandular syndrome type 1 (APS1) complicated by severe vascular insufficiency due to diffuse vascular calcification. APS1 is characterised clinically by multiple autoimmune conditions and development of at least two components of the triad of mucocutaneous candidiasis, hypoparathyroidism, and autoimmune adrenal insufficiency. We highlight the problems in current serum calcium monitoring methods and suggest that fluctuations in serum calcium concentrations due to difficulties treating hypoparathyroidism may have contributed to the vascular calcification seen in this case. (Source: Infectious Diseases in Obstetrics and Gynecology)

Diagnosis of adrenal failure in critically ill patients

Arquivos Brasileiros de Endocrinologia e Metabologia — Sat, 03 Sep 2011 15:39:34 +0100

In the last two decades there was important evolution on the knowledge of the function of the hypothalamic-pituitary-adrenal axis. In the last decade, the expression "relative adrenal insufficiency" (RAI) was created, and more recently "critical illness-related corticosteroid insufficiency" (CIRCI) was used to designate those patients in which cortisol production was not sufficiently increased in stress situations. Patients with CIRCI have elevated hospital morbidity and mortality. Currently, there is a wide discussion about diagnostic criteria for this dysfunction. Besides basal cortisol, some publications now study the role of other tests, such as cortrosyn test - either in low (1 μg) or high doses (250 μg); free cortisol, salivary cortisol, metyrapone test and others. With this review...

A case of adrenal insufficiency secondary to syphilis and difficulties in the diagnostic work‐up

Australasian Journal of Dermatology — Thu, 01 Sep 2011 23:00:00 +0100

We report a case of Addison's disease presumed to be secondary to syphilis. The patient presented with adrenal failure and a maculopapular rash on his trunk and palms. Syphilis was suspected but the non‐treponemal serological test used (the rapid plasma reagin test (RPR)) was falsely negative due to the prozone phenomenon. Treatment with benzathine penicillin resulted in normalization of adrenal function and resolution of the cutaneous findings. Repeat testing of the previously obtained serum sample showed the RPR to be positive on increasing dilutions of serum, consistent with the prozone effect. (Source: Australasian Journal of Dermatology)

Variceal Bleeding in Cirrhotic Patients Is Associated With Adrenal Dysfunction: What Is the Evidence?

Clinical Gastroenterology and Hepatology — Thu, 01 Sep 2011 04:00:00 +0100

We read the article by Triantos and colleagues with great interest. The authors prospectively assessed adrenal function in 20 patients with variceal bleeding and 60 nonbleeding cirrhotic patients with a low-dose short synacthen test (LD-SST; 1 μg) and/or a standard-dose (250 μg) short synacthen test. Their crude conclusion was that adrenal insufficiency in bleeding patients using well-defined serum total cortisol thresholds was common, mainly using the low-dose short synacthen test (60% vs 48% in nonbleeding patients). The authors also found no significant difference in albumin concentrations between bleeding patients with and without adrenal insufficiency. These interesting results deserve comment because nearly 80% of circulating cortisol in serum is bound to corticosteroid-binding glo...

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A Novel Entity of Clinically Isolated Adrenal Insufficiency Caused by a Partially Inactivating Mutation of the Gene Encoding for P450 Side Chain Cleavage Enzyme (CYP11A1).

The Journal of Clinical Endocrinology and Metabolism — Tue, 30 Aug 2011 23:00:00 +0100

Conclusions:We provide the first evidence that partial CYP11A1 deficiency has to be considered as a differential diagnosis in clinically isolated adrenal insufficiency. Our assays demonstrate a tighter genotype-phenotype correlation in CYP11A1 deficiency than previous in vitro studies. PMID: 21880796 [PubMed - as supplied by publisher] (Source: The Journal of Clinical Endocrinology and Metabolism)

What Causes Increased Body Hair?

PediatricEducation.org — Mon, 29 Aug 2011 00:21:30 +0100

Discussion Body hair, including its amount and distribution, have cultural norms and social implications that vary according to the group and individual. There are entire industries which cater to removing hair in certain body parts (i.e. underarms and legs for western women) and adding hair in other locations (i.e. head for western men). Excessive body hair (relatively) is usually often due to normal physiologic process (such as puberty) or genetic. Two definitions which are often confused are: Hypertrichosis – excessive, non-androgen induced hair growth in a localized or generalized pattern distribution in males or females. Hirsuitism – excessive, androgen-induced hair growth in a male pattern distribution in children and women. Learning Point The differential diagnosis of ...

Adrenal hemorrhage in newborns: a retrospective study.

World Journal of Pediatrics : WJP — Fri, 26 Aug 2011 23:00:00 +0100

CONCLUSIONS: AH occurs in the newborns with unexplained jaundice. Adrenal insufficiency is more frequent in preterm than in mature infants. Abdominal USG is required to determine AH in a newborn with swelling and bluish discoloration of the scrotum. Serial USG is the best modality for monitoring AH to prevent unnecessary surgery. PMID: 21874621 [PubMed - as supplied by publisher] (Source: World Journal of Pediatrics : WJP)

A pilot study of adrenal suppression after dexamethasone therapy as an antiemetic in cancer patients

Supportive Care in Cancer — Thu, 18 Aug 2011 06:03:23 +0100

Conclusions We suggest that suppression of adrenal response is common after antiemetic dexamethasone therapy in cancer patients receiving chemotherapy. Content Type Journal ArticleCategory Original ArticlePages 1-8DOI 10.1007/s00520-011-1248-zAuthors Hye-Suk Han, Department of Internal Medicine, Chungbuk National University College of Medicine, 410 Seongbong-ro, Heungduk-gu, Cheongju 361-711, South KoreaYoung Kwang Shim, Department of Internal Medicine, Chungbuk National University College of Medicine, 410 Seongbong-ro, Heungduk-gu, Cheongju 361-711, South KoreaJeong Eun Kim, Department of Internal Medicine, Chungbuk National University College of Medicine, 410 Seongbong-ro, Heungduk-gu, Cheongju 361-711, South KoreaHyun-Jung Jeon, Department of Internal Medicine, Ch...

Assessing adrenal insufficiency of corticosteroid secretion using free versus total cortisol levels in critical illness

Intensive Care Medicine — Thu, 18 Aug 2011 06:01:09 +0100

Conclusions Although the biologically active free cortisol fraction depends on binding proteins, total cortisol correlates to free cortisol in treatment-insensitive hypotension during critical illness. In sepsis, albumin is not an important binding molecule. Subnormal increments in total cortisol upon ACTH suffice in assessing relative adrenal insufficiency, particularly in sepsis. Content Type Journal ArticleCategory OriginalPages 1-8DOI 10.1007/s00134-011-2342-xAuthors Nienke Molenaar, Department of Intensive Care, Vrije Universiteit Medical Center, De Boelelaan 1117, 1081 HV Amsterdam, The NetherlandsA. B. Johan Groeneveld, Department of Intensive Care, Vrije Universiteit Medical Center, De Boelelaan 1117, 1081 HV Amsterdam, The NetherlandsHilde M. Dijstelbloem, ...

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The pituitary stalk transection syndrome: multifaceted presentation in adulthood

Pituitary — Mon, 08 Aug 2011 19:53:14 +0100

We present 4 cases of stalk transection syndrome diagnosed by the adult endocrinologist that reflect its pleiotropic manifestations. In all cases, MRI showed pathognomonic findings with small anterior pituitary, diminutive or absent infundibulum and ectopic posterior pituitary at the median eminence. Clinical presentation occurred in childhood or the second decade of life. The hormonal deficits were variable in severity and onset, with adrenal insufficiency diagnosed in the second and forth decade in three patients, and absent in another. Growth hormone deficiency was diagnosed before age 10 in three cases and at age 20 in one case with normal spontaneous linear growth. Hypothyroidism had onset in the first or second decade of life and hypogonadism was diagnosed during work-up for l...

Meeting highlights from the Committee for Medicinal Products for Human Use (CHMP) 18-21 July 2011 (European Medicines Agency press release, 22 July 2011)

Society for Endocrinology — Mon, 25 Jul 2011 12:00:00 +0100

Highlights of the latest meeting of the European Medicines Agency Committee for Medicinal Products for Human Use are now available online, including a conclusion of their review into the benefit-risk profile of pioglitazone, approval of marketing authorisation for a new drug for adrenal insufficiency, and a note on the current supply shortage of Thyrogen. Full press release (Source: Society for Endocrinology)

The Use of Oral Budesonide in Adolescents and Adults With Protein-Losing Enteropathy After the Fontan Operation.

The Annals of Thoracic Surgery — Thu, 21 Jul 2011 23:00:00 +0100

CONCLUSIONS: CR-budesonide can be used to treat PLE in certain patients, but careful assessment of hepatic function should be performed before initiation of therapy as systemic side effects can limit treatment. Normal serum liver function tests do not preclude hepatic dysfunction in the Fontan patient, and it is important to perform radiographic assessments as well. PMID: 21784410 [PubMed - as supplied by publisher] (Source: The Annals of Thoracic Surgery)

Clinical approach to adrenal insufficiency in hospitalised patients

International Journal of Clinical Practice — Thu, 21 Jul 2011 21:50:41 +0100

This article extensively reviews various aetiologies and management of adrenal insufficiency. (Source: International Journal of Clinical Practice)

Pseudotumor cerebri during Cushing's disease treatment with ketoconazole

Arquivos Brasileiros de Endocrinologia e Metabologia — Sat, 16 Jul 2011 16:49:17 +0100

We describe here the case of a 12-year old girl with CD who developed benign intracranial hypertension during treatment with ketoconazole. She presented headache, vomiting, a black spot on her right temporal visual field, and signs of elevated intracranial pressure. Pituitary image was normal on magnetic resonance image (MRI), and all symptoms improved after treatment with acetazolamide. We call attention to the diagnosis of this disorder in CD patients, especially children on ketoconazole treatment, because it could be confounded with adrenal insufficiency and lead to definitive severe visual impairment.Hipertensão intracraniana benigna (Pseudotumor cerebral) tem sido descrita relacionada à redução dos níveis de esteroides séricos na doença de Cushing (DC), especialmente após a re...

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Endothelial dysfunction: a critical determinant in inflammation‐associated adrenal insufficiency?

European Journal of Clinical Investigation — Thu, 14 Jul 2011 06:45:49 +0100

(Source: European Journal of Clinical Investigation)

Methylprednisolone/opioid analgesics: Central adrenal insufficiency: case report

Reactions — Wed, 13 Jul 2011 16:51:36 +0100

(Source: Reactions)

Valproic acid: Adrenal insufficiency: case report

Reactions — Wed, 13 Jul 2011 16:51:36 +0100

(Source: Reactions)

Lopinavir-Ritonavir May Alter Adrenal Function in NeonatesLopinavir-Ritonavir May Alter Adrenal Function in Neonates

Medscape Hiv-Aids Headlines — Thu, 07 Jul 2011 01:47:54 +0100

Treatment of newborns with the protease inhibitor lopinavir-ritonavir may lead to adrenal dysfunction, including life-threatening adrenal insufficiency in premature infants, researchers report. Reuters Health Information (Source: Medscape Hiv-Aids Headlines)

Association of Prenatal and Postnatal Exposure to Lopinavir-Ritonavir and Adrenal Dysfunction Among Uninfected Infants of HIV-Infected Mothers [Preliminary Communication]

JAMA — Mon, 04 Jul 2011 23:00:00 +0100

Conclusion Among newborn children of HIV-1–infected mothers exposed in utero to lopinavir-ritonavir, postnatal treatment with a lopinavir-ritonavir–based regimen, compared with a zidovudine-based regimen, was associated with transient adrenal dysfunction. (Source: JAMA)

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Lopinavir-ritonavir may alter adrenal function in neonates

Modern Medicine — Mon, 04 Jul 2011 23:00:00 +0100

NEW YORK (Reuters Health) - Treatment of newborns with the protease inhibitor lopinavir-ritonavir may lead to adrenal dysfunction, including life-threatening adrenal insufficiency in premature infants, researchers report. (Source: Modern Medicine)

Triamcinolone: Adrenal insufficiency following epidural administration: case report

Reactions — Sun, 03 Jul 2011 18:34:01 +0100

(Source: Reactions)

Low dose hydrocortisone treatment for patients with septic shock: a pilot study comparing three days with seven days

Respirology — Thu, 30 Jun 2011 23:00:00 +0100

Outcomes of Etomidate in Severe Sepsis and Septic Shock: Dmello D, Taylor S, O'Brien J, et al. Chest 2010;138:1327–32.

The Journal of Emergency Medicine — Thu, 30 Jun 2011 23:00:00 +0100

The discussion over transient adrenal suppression caused by single-dose etomidate has been heated in recent years. The aim of this study was to evaluate the effect of single-dose etomidate in patients with severe sepsis and septic shock. This was a retrospective cohort study conducted in a 56-bed medical/surgical intensive care unit (ICU) at St. John's Mercy Medical Center in St. Louis, Missouri. The study evaluated 224 consecutive patients who had severe sepsis or septic shock and required intubation and mechanical ventilation comparing those patients who had received etomidate to those who had not. Outcome measures were not explicitly defined, but data recorded included Acute Physiology and Chronic Health Evaluation (APACHE) II and Mortality Probability Model at zero hour (MPM-II0) scor...

Hydrocortisone Therapy for Patients with Multiple Trauma: The Randomized Controlled HYPOLYTE Study: Roquilly A, Mahe PJ, Seguin P, et al. JAMA 2011;305:1201–9.

The Journal of Emergency Medicine — Thu, 30 Jun 2011 23:00:00 +0100

Stress-related adrenal insufficiency associated with polytrauma has been postulated as a factor in the development of hospital-acquired infections. This multicenter, randomized, double-blind, parallel, placebo-controlled study conducted in seven separate intensive care units (ICUs) in France from November 15, 2006 to August 4, 2009 was designed to test the idea that steroid treatment could reduce the prevalence of hospital-acquired pneumonia in trauma patients. All multiple-trauma patients, defined as having two or more traumatic injuries and injury severity score>15, aged>15 years who were expected to require mechanical ventilation for more than 48h, were included. All patients were tested for corticosteroid insufficiency with a corticotropin test. Upon completion of this test, patients w...

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Official Positions for FRAX® Clinical Regarding Glucocorticoids: The impact of the Use of Glucocorticoids on the Estimate by FRAX® of the 10 Year Risk of Fracture: From Joint Official Positions Development Conference of the International Society for Clinical Densitometry and International Osteoporosis Foundation on FRAX®

Journal of Clinical Densitometry — Thu, 30 Jun 2011 23:00:00 +0100

Abstract: Given the significant impact the use of glucocorticoids can have on fracture risk independent of bone density, their use has been incorporated as one of the clinical risk factors for calculating the 10-year fracture risk in the World Health Organization’s Fracture Risk Assessment Tool (FRAX®). Like the other clinical risk factors, the use of glucocorticoids is included as a dichotomous variable with use of steroids defined as past or present exposure of 3 months or more of use of a daily dose of 5 mg or more of prednisolone or equivalent. The purpose of this report is to give clinicians guidance on adjustments which should be made to the 10-year risk based on the dose, duration of use and mode of delivery of glucocorticoids preparations. A subcommittee of the International Soc...

Endoscopic Transsphenoidal Surgery for Acromegaly: Remission Using Modern Criteria, Complications, and Predictors of Outcome.

The Journal of Clinical Endocrinology and Metabolism — Tue, 28 Jun 2011 23:00:00 +0100

Conclusions: ETSS for GH adenomas is associated with high rates of remission and a low incidence of new endocrinopathy. Despite the panoramic views offered by the endoscope, invasive tumors continue to have lower rates of remission. PMID: 21715544 [PubMed - as supplied by publisher] (Source: The Journal of Clinical Endocrinology and Metabolism)

Rapid Onset of Iatrogenic Adrenal Insufficiency in a Patient with Cystic Fibrosis-Related Liver Disease Treated with Inhaled Corticosteroids and a Moderate CYP3A4 Inhibitor (July/August).

The Annals of Pharmacotherapy — Mon, 27 Jun 2011 23:00:00 +0100

CONCLUSIONS CYP3A4-mediated drug interactions represent a significant risk in patients treated with long-term inhaled corticosteroids. The presence of clinically significant CF-related liver disease may enhance this risk. PMID: 21712512 [PubMed - as supplied by publisher] (Source: The Annals of Pharmacotherapy)

Adrenocorticotropic hormone and cortisol response to corticotropin releasing hormone in the critically ill—a novel assessment of the hypothalamic-pituitary-adrenal axis

American Journal of Surgery — Fri, 17 Jun 2011 04:00:00 +0100

Conclusions: ACTH responsiveness was increased in nonsurvivors and may predict mortality. (Source: American Journal of Surgery)

Neurological features in adult Triple-A (Allgrove) syndrome

Journal of Neurology — Thu, 09 Jun 2011 05:48:31 +0100

Abstract Triple-A or Allgrove syndrome is a rare multisystem disease classically associated with esophageal achalasia, adrenal insufficiency and alacrima. Here, we describe the poorly understood neurological characteristics often associated with this condition, through the clinical and electrophysiological analysis of eight patients. All patients were genetically confirmed and had a mutation in the ALADIN gene. They all displayed a classical picture of Triple-A syndrome: all suffered from achalasia and alacrima and half of them from adrenal insufficiency. However, all harbored a neurological picture characterized by a recognizable pattern of peripheral neuropathy. Other neurological features included cognitive deficits, pyramidal syndrome, cerebellar dysfunction, dysautono...

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Low prevalence of hypopituitarism after traumatic brain injury - a multi-center study.

European Journal of Endocrinology — Sun, 05 Jun 2011 23:00:00 +0100

Conclusion: In this study, the prevalence of hypopituitarism during long-term follow-up after TBI was low. Prospective studies are urgently needed to find reliable predictive tools for the identification of patients with a significant pre-test likelihood for hypopituitarism after TBI. PMID: 21646286 [PubMed - as supplied by publisher] (Source: European Journal of Endocrinology)

Partial Deletion of the NR5A1 (SF1) Gene Detected by Synthetic Probe MLPA in a Patient with XY Gonadal Disorder of Sex Development.

Thu, 02 Jun 2011 23:00:00 +0100

Authors: Barbaro M, Cools M, Looijenga LH, Drop SL, Wedell A Steroidogenic factor 1 (SF1, officially NR5A1) is a nuclear receptor involved in adrenal and gonadal development. NR5A1 mutations have been identified in patients with various forms of 46,XY disorders of sex development (DSD), including complete gonadal dysgenesis with or without adrenal insufficiency, mild testicular dysgenesis with ambiguous external genitalia or female external genitalia with clitoromegaly, and penoscrotal hypospadias. We developed a synthetic probe set for MLPA analysis of the NR5A1 gene covering its 7 exons and analyzed 20 patients with 46,XY gonadal DSD in whom analyses failed to identify a genetic cause. We identified a partial NR5A1 deletion affecting exons 2 and 3, leading to NR5A1 haploinsufficiency...

Functional effects of DAX-1 mutations identified in patients with X-linked adrenal hypoplasia congenita

Metabolism - Clinical and Experimental — Wed, 01 Jun 2011 04:00:00 +0100

In this study, we investigated the functional defects of DAX-1 caused by mutations identified in 3 unrelated Korean patients with adrenal hypoplasia congenita. The DAX-1 gene was directly sequenced using genomic DNA isolated from peripheral blood leukocytes. The functional defects of DAX-1 caused by mutations were evaluated using an in vitro promoter assay. After mutagenesis of DAX-1 complementary DNA in the pcDNA3.1 vector, steroidogenic factor 1 and the promoter region of steroidogenic acute regulatory protein (StAR) genes in pGL4.10[luc2] were transiently cotransfected into human embryonic kidney 293 cells, followed by luminometry measurements of the luciferase activity of StAR. Mutation analysis of 3 patients revealed p.L386delfsX2, p.W105X, and p.Q252X mutations of the DAX-1 gene. The...

Adrenocortical cell transplantation reverses a murine model of adrenal failure

Journal of Pediatric Surgery — Tue, 31 May 2011 23:00:00 +0100

Conclusion: Cells transplantation restored the adrenocortical function in these mice. Further optimization of this technique could bring a curative therapy to patients with adrenal insufficiency. (Source: Journal of Pediatric Surgery)

Congenital Adrenal Hyperplasia

Journal of Pediatric and Adolescent Gynecology — Wed, 25 May 2011 16:45:28 +0100

This article will review key aspects regarding pathophysiology, diagnosis, and treatment of CAH. (Source: Journal of Pediatric and Adolescent Gynecology)

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Natural Killer T-Cell Lymphoma Presenting As Adrenal Mass and Partial Adrenal Insufficiency Followed by CNS Involvement [DIAGNOSIS IN ONCOLOGY]

Journal of Clinical Oncology — Tue, 17 May 2011 23:00:00 +0100

(Source: Journal of Clinical Oncology)

Pediatric Adrenal Trauma in the 21st Century: Children's Hospital of Atlanta Experience

The Journal of Urology — Sun, 15 May 2011 23:00:00 +0100

Conclusions: Adrenal trauma in children is rare. Although typically associated with high morbidity, this outcome is likely from related injuries as an isolated adrenal injury generally portends a benign course. (Source: The Journal of Urology)

Medical Management of Patients With Brain Tumors

Current Treatment Options in Neurology — Tue, 10 May 2011 05:56:59 +0100

Opinion statement Patients with brain tumors require meticulous attention to medical issues resulting from their disease or its therapy. The following specific issues are the ones most frequently arising in the purview of neurologists: (1) Vasogenic edema: Corticosteroids should be used in divided doses in the minimum amount required to control symptoms and should be tapered as quickly as possible. Some patients may require long-term steroid supplementation, and symptoms of adrenal insufficiency should be investigated with 8 am cortisol measurement and treated with appropriate repletion. (2) Seizures: Patients with brain tumors should receive antiepileptic drugs only if they have had seizures, and the drugs should be chosen to minimize cognitive effects and interactions wi...

Adrenal Insufficiency with Gingival Mass—An Unusual Presentation of Hepatocellular Carcinoma

Journal of Oral and Maxillofacial Surgery — Sun, 08 May 2011 23:00:00 +0100

We report a case with clinical, radiologic, biochemical, and histopathologic evidence of metastases of HCC, with the initial presentation a solitary gingival mass. Subsequent examination revealed adrenal insufficiency. A complete workup confirmed the presence of multiple organ involvement, including metastasis to the adrenal glands. (Source: Journal of Oral and Maxillofacial Surgery)

Growth Hormone Deficiency, Short Stature, and Juvenile Rheumatoid Arthritis in a Patient with Autoimmune Polyglandular Syndrome Type 1: Case Report and Brief Review of the Literature

Journal of Oncology — Thu, 05 May 2011 14:35:30 +0100

We report the chronological history of a female patient who presented with features most consistent with APS type 1, along with growth hormone deficiency and juvenile rheumatoid arthritis (JRA). In terms of her autoimmune diagnoses, she first presented with JRA at three years of age, then hypocalcemia and hypoparathyroidism at five years of age, type 1 diabetes (DM 1) at age eleven years, adrenal insufficiency at age fourteen years, recurrent mucocutaneous candidiasis as a teenager, growth hormone deficiency at age fourteen years leading to significant short stature, primary amenorrhoea, and hypogonadism, and finally alopecia at age twenty-six years. In addition to this, she has suffered other nonautoimmune medical problems including a Tetralogy of Fallot with a surgical repair at age six ...

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Adrenal insufficiency uncommon after transsphenoidal surgery for acromegaly

Modern Medicine — Tue, 03 May 2011 23:00:00 +0100

NEW YORK (Reuters Health) - Long-term rates of adrenal insufficiency are low after transsphenoidal surgery for growth hormone-secreting pituitary adenomas, researchers from the Netherlands report in the April 6th online issue of The Journal of Clinical Endocrinology & Metabolism. (Source: Modern Medicine)

Phase II Trial of Saracatinib (AZD0530), an Oral SRC-inhibitor for the Treatment of Patients with Hormone Receptor-negative Metastatic Breast Cancer.

Clinical Genitourinary Cancer — Mon, 02 May 2011 23:00:00 +0100

CONCLUSIONS: These efficacy results were not sufficiently promising to justify continued accrual to this study. Based on this series, saracatinib does not appear to have significant single-agent activity for the treatment of patients with ER(-)/PR(-) MBC. PMID: 21729667 [PubMed - as supplied by publisher] (Source: Clinical Genitourinary Cancer)

Relative Adrenal Insufficiency in the Critical Care Setting: Debunking the Classic Myth

World Journal of Surgery — Sun, 01 May 2011 15:32:25 +0100

Conclusion Hyponatremia, hyperkalemia, hypercalcemia, and hypoglycemia do not predominate in RAI and laboratory values are of minimal value in predicting patients with RAI. Content Type Journal ArticlePages 1-6DOI 10.1007/s00268-011-1126-3Authors Fatuma Kromah, Department of Surgery, Texas Tech University Health Sciences Center, Paul L. Foster School of Medicine, 4800 Alberta Ave., El Paso, TX 79905, USAAlan Tyroch, Department of Surgery, Texas Tech University Health Sciences Center, Paul L. Foster School of Medicine, 4800 Alberta Ave., El Paso, TX 79905, USASusan McLean, Department of Surgery, Texas Tech University Health Sciences Center, Paul L. Foster School of Medicine, 4800 Alberta Ave., El Paso, TX 79905, USAHarold Hughes, Department of Internal Medicine, Texas...

Hormonal disturbances in visceral leishmaniasis (kala-azar).

The American Journal of Tropical Medicine and Hygiene — Sat, 30 Apr 2011 23:00:00 +0100

This study presents a cross-sectional analysis of the hormonal alterations of patients with visceral leishmaniasis. The diagnosis was established by the bone marrow aspiration and polymerase chain reaction test. Primary adrenal insufficiency was observed in 45.8% of patients; low aldosterone/renin plasma ratio in 69.4%; low daily urinary aldosterone excretion in 61.1%; and low transtubular potassium gradient in 68.0%. All patients had normal plasma antidiuretic hormone (ADH) concentrations, hyponatremia, and high urinary osmolality. Plasma parathyroid hormone was low in 63%; hypomagnesemia was present in 46.4%, and increased Mg(++)(EF) in 100%. Primary thyroid insufficiency was observed in 24.6%, and secondary thyroid insufficiency in 14.1%. Normal follicle-stimulating hormone plasma level...

When Normal Is Abnormal: Keys to Laboratory Diagnosis of Hidden Endocrine Disease

Topics in Companion Animal Medicine — Sat, 30 Apr 2011 23:00:00 +0100

Although veterinary clinicians commonly rely on panels of laboratory tests with individual results flagged when abnormal, care should be taken in interpreting normal test results as well. There are several examples of this in evaluating patients with endocrine disease. The finding of a normal leukogram (absence of a stress leukogram) can be indicative of adrenal insufficiency in dogs, and this disorder can be especially elusive when there are no overt indicators of mineralocorticoid deficiency. Cats with hyperthyroidism can have normal serum thyroid hormone concentrations, normal hematocrits, and normal serum concentrations of creatinine despite the presence of disease that affects these parameters. A normal serum phosphorus concentration, in the face of azotemia, isosthenuria, and hyperte...

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Measuring adrenal autoantibody response: Interlaboratory concordance in the first international serum exchange for the determination of 21-hydroxylase autoantibodies.

Clinical Immunology — Wed, 27 Apr 2011 23:00:00 +0100

Authors: Falorni A, Chen S, Zanchetta R, Yu L, Tiberti C, Bacosi ML, Furmaniak J, Bini V, Dotta F, Eisenbarth GS, Rees Smith B, Betterle C 21-hydroxylase autoantibodies (21OHAb) are the gold standard immune marker to identify patients with clinical or subclinical autoimmune Addison's disease (AAD). No assessment of interlaboratory concordance has been made for 21OHAb measurement. Serum samples from 267 patients with primary adrenal insufficiency and from 83 healthy control subjects were distributed to four independent laboratories that determined presence and titer of 21OHAb, by using radiobinding assays with either in vitro translated (35)S-radiolabelled or (125)I-radiolabelled autoantigen. Cohen's κ of inter-rater agreement ranged from 0.857 to 0.983, showing a very good concordance...

Budesonide: Adrenal insufficiency in an elderly patient: case report

Reactions — Sun, 24 Apr 2011 16:50:04 +0100

(Source: Reactions)

SfE launches new patient information leaflet on adrenal insufficiency

Society for Endocrinology — Tue, 19 Apr 2011 12:00:00 +0100

The Society for Endocrinology has produced a new patient information leaflet on adrenal insufficiency. This leaflet provides an introduction to the causes and symptoms of adrenal insufficiency and the tests used to diagnose this condition. It is endorsed by the Addison’s Disease Self-Help Group, the European Society of Endocrinology, the Pituitary Foundation and the Royal College of Physicians. A pdf copy is available via the link below. If you would like any hard copies of the leaflet, please email public@endocrinology.org. Adrenal insufficiency leaflet (pdf) (Source: Society for Endocrinology)

Primary antiphospholipid antibody syndrome presenting with adrenal insufficiency in a child: case report and review of literature.

Lupus — Mon, 18 Apr 2011 23:00:00 +0100

Authors: Bhakhri BK, Katewa S, Sharma R, Mahajan S A 7-year-old boy presented with adrenal insufficiency. He subsequently developed venous thrombosis in the limbs and was diagnosed with primary antiphospholipid syndrome (PAPS) based on clinical and laboratory parameters. Both adrenals were normal on imaging. He required thrombolysis and anticoagulation. The progressive course of PAPS was controlled with methylprednisolone. There are few reports of PAPS in pediatric patients, and associated adrenal involvement is rare. The unusual presentation, course and management of the patient and of four other reported children with adrenal insufficiency heralding manifestation of PAPS are discussed. PMID: 21505010 [PubMed - as supplied by publisher] (Source: Lupus)

Clinical and MRI characteristics of acute encephalopathy in congenital adrenal hyperplasia

Journal of the Neurological Sciences — Thu, 14 Apr 2011 23:00:00 +0100

We report three CAH children with acute infection-related encephalopathy. They exhibited disturbed consciousness or seizures, which did not improve after glucocorticoid administration, accompanied by clinical and laboratory findings of adrenal insufficiency. Brain MRI disclosed various patterns of white matter lesions, suggesting different types of acute encephalopathy such as clinically mild encephalitis/encephalopathy with a reversible splenial lesion or hemiconvulsion-hemiplegia syndrome. Acute encephalopathy should be considered and brain MRI immediately performed when impairment of consciousness does not improve after intravenous glucocorticoid administration in CAH patients. Further research is required to elucidate the epidemiology and pathogenic mechanisms of acute encephalopathy i...

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Critical Illness-Related Corticosteroid Insufficiency in Patients With Cirrhosis and Variceal Bleeding

Clinical Gastroenterology and Hepatology — Sun, 10 Apr 2011 23:00:00 +0100

Background & Aims: Relative adrenal insufficiency (AI) occurs in patients with cirrhosis with sepsis, but not with variceal bleeding. We evaluated adrenal function in cirrhotic patients with and without bleeding.Methods: Twenty cirrhotic patients with variceal bleeding were evaluated using the short synacthen test (SST) and 10 using the low-dose synacthen test (LDSST) followed by SST. The control group included 60 stable cirrhotic patients, assessed by LDSST (n = 50) or SST (n = 10), and 14 healthy volunteers. AI was diagnosed using SST, based on peak cortisol levels ≤18 μg/dL in nonstressed patients or Δmax (Source: Clinical Gastroenterology and Hepatology)

Presentation of Primary Adrenal Insufficiency in Childhood.

The Journal of Clinical Endocrinology and Metabolism — Tue, 05 Apr 2011 23:00:00 +0100

Conclusions: Hyperkalemia is not a consistent presenting sign of primary adrenal insufficiency in childhood, and its absence cannot rule out this condition. A combination of chronic or subacute clinical symptoms, hypotension, and hyponatremia should raise suspicion of adrenal insufficiency. PMID: 21470994 [PubMed - as supplied by publisher] (Source: The Journal of Clinical Endocrinology and Metabolism)

Low Incidence of Adrenal Insufficiency after Transsphenoidal Surgery in Patients with Acromegaly: A Long-Term Follow-Up Study.

The Journal of Clinical Endocrinology and Metabolism — Tue, 05 Apr 2011 23:00:00 +0100

Conclusion: The prevalence of adrenal insufficiency 1 yr after surgery was 9%, whereas during prolonged follow-up, the incidence rate of adrenal insufficiency was only 2/1000 person-years in patients in remission after surgery. Therefore, development of late-onset adrenal insufficiency is a very infrequent complication in patients with acromegaly in remission after transsphenoidal surgery only. PMID: 21470989 [PubMed - as supplied by publisher] (Source: The Journal of Clinical Endocrinology and Metabolism)

Genetic variation of TLR4 influences immunoendocrine stress response: an observational study in cardiac surgical patients

Critical Care — Mon, 04 Apr 2011 23:00:00 +0100

Conclusions: Regulation of the immunoendocrine stress response during systemic inflamma-tion is influenced by the presence of a TLR4 SNP. Cardiac surgical patients carrying this ge-notype showed decreased serum concentrations of ACTH, IL-8, IL-10 and GM-CSF. This finding might have impact on interpreting previous and designing future trials on diagnosing and modulating immunoendocrine dysregulation (e.g. adrenal insufficiency) during systemic inflammation and sepsis. (Source: Critical Care)

Clobetasol: Cushing syndrome and adrenal insufficiency in an infant: case report

Reactions — Mon, 04 Apr 2011 16:58:12 +0100

(Source: Reactions)

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Diabetic cystopathy: do we recognise this in our patients?

Practical Diabetes International — Thu, 31 Mar 2011 23:00:00 +0100

AbstractA 44‐year‐old South Asian woman, with type 2 diabetes requiring insulin, presented with multiple syncopal episodes. Her diabetes was complicated by peripheral neuropathy, diabetic retinopathy and nephropathy. She also had features of autonomic neuropathy. Short synacthen test ruled out adrenal insufficiency; thyroid function was normal. HbA1c was elevated at 14.6% (136mmol/mol). Abdominal computed tomography showed grossly dilated bladder (9.5cm x 14cm x 17.5cm), compressing the mid‐ureter. The size suggested an on‐going chronic process, consistent with diabetic cystopathy.An indwelling urethral catheter relieved the bladder distension and the patient was later successfully educated to void the bladder by the clock rather than bladder sensation. Euglycaemia was achieved wit...

Comparison of low‐dose and high‐dose cosyntropin stimulation testing in children

Pediatrics International — Thu, 31 Mar 2011 23:00:00 +0100

Conclusions: LD cosyntropin stimulation testing results should be interpreted cautiously when used alone to prevent unnecessary long‐term treatment. Using a lower cut‐off for LD (≥14 µg/dL) seems to avoid false positive results and still detects most cases of impaired HPAA. (Source: Pediatrics International)

RCT: Hydrocortisone reduces pneumonia rates in severe trauma patients

NeLM - News — Wed, 23 Mar 2011 00:00:00 +0100

Source: JAMA Area: News 'Stress dose' hydrocortisone reduced the incidence of pneumonia compared to placebo in ITU patients with severe trauma, in a controlled trial. Pneumonia is a common problem in patients with severe trauma, and increases the risk of adverse clinical outcomes. There is evidence that 'stress-dose' hydrocortisone could reduce the risk of pneumonia in such patients, and this trial was intended to determine whether it did so. Participants were patients admitted to one of 7 French ITU with multiple trauma (injury severity score >15), aged over 15.25 years, and expected to require ventilation for at least 48 hours. Major exclusion criteria were recent treatment with corticosteroids, adrenal insufficiency, and immunosuppression. They were randomised to receive ...

Adrenocortical Insufficiency in Horses and Foals

Veterinary Clinics of North America: Equine Practice — Tue, 15 Mar 2011 22:52:40 +0100

The adrenal cortices produce various steroid hormones that play vital roles in several physiologic processes. Although permanent adrenocortical insufficiency is rare in all species, emerging evidence in both human and equine medicine suggests that transient reversible adrenocortical dysfunction resulting in cortisol insufficiency frequently develops during critical illness. This syndrome is termed relative adrenal insufficiency (RAI) or critical illness–related corticosteroid insufficiency (CIRCI) and can contribute substantially to morbidity and mortality associated with the primary disease. This review discusses the mechanisms, diagnosis, and clinical consequences of adrenocortical insufficiency, with particular focus on the current understanding of RAI/CIRCI in horses and foals. (Sour...

Catecholamine-resistant shock and hypoglycemic coma after cardiotomy in a patient with unexpected isolated ACTH deficiency

Journal of Anesthesia — Sat, 12 Mar 2011 16:53:14 +0100

Abstract Isolated adrenocorticotropic hormone (ACTH) deficiency is an extremely rare disease in which ACTH-producing cells of the pituitary gland are selectively damaged. The resulting decline in ACTH production and secretion results in chronic secondary adrenocortical insufficiency. The patient in this case did not present with adrenal insufficiency symptoms prior to surgery. However, after cardiotomy under extracorporeal circulation, the patient lapsed into a catecholamine-resistant shock and hypoglycemic coma. Acute adrenal insufficiency was strongly suspected, and the patient was diagnosed with isolated ACTH deficiency after careful examination. Because the demand for cortisol increases after highly invasive surgeries, cortisol supplementation therapy is essential for ...

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Etomidate use in severe sepsis and septic shock patients does not contribute to mortality

Internal and Emergency Medicine — Fri, 11 Mar 2011 06:51:48 +0100

Abstract Use of etomidate in severe sepsis and septic shock has been challenged in recent literature due to its link to adrenal insufficiency and suspected increased mortality. We hypothesized that etomidate does not contribute to mortality in this patient population. A retrospective chart review of 230 intubated, severe sepsis/septic shock patients at two university tertiary care referral centers was conducted for patients receiving treatment between 12/2001 and 10/2009. The primary endpoint was in-hospital mortality. Additional investigated variables included the use of corticosteroids, hospital and intensive care unit (ICU) length of stay, mechanical ventilation days and patient demographics. One hundred seventy-three patients received etomidate and fifty-seven patients...

The effect of etomidate on adrenal function in critical illness: a systematic review

Intensive Care Medicine — Fri, 04 Mar 2011 10:51:17 +0100

Conclusions There is an increased rate of AI and mortality in critically ill patients who received etomidate. Content Type Journal ArticlePages 1-10DOI 10.1007/s00134-011-2160-1Authors Stewart G. Albert, Division of Endocrinology, Department of Internal Medicine, Saint Louis University School of Medicine, 1402 South Grand Blvd, St. Louis, MO 63104, USASrividya Ariyan, Division of Endocrinology, Department of Internal Medicine, Saint Louis University School of Medicine, 1402 South Grand Blvd, St. Louis, MO 63104, USAAyesha Rather, Division of Endocrinology, Department of Internal Medicine, Saint Louis University School of Medicine, 1402 South Grand Blvd, St. Louis, MO 63104, USA Journal Intensive Care MedicineOnline ISSN 1432-1238Print ISSN 0342-4642 (Source: Inte...

Primary Adrenal Lymphoma: 3 Case Reports with Different Outcomes

Experimental and Clinical Endocrinology and Diabetes — Thu, 03 Mar 2011 00:00:00 +0100

Exp Clin Endocrinol DiabetesDOI: 10.1055/s-0031-1271629AbstractPrimary adrenal lymphoma (PAL) is an extremely rare entity, with approximately 70 cases reported in the English literature and 120 cases worldwide. Here we report the cases of a 53-year-old and a 62-year-old male patient and a 60-year-old female patient affected by large B-cell non-Hodgkin lymphoma of the adrenal gland. We summarize the diagnostic approaches that confirmed the diagnosis of PAL and describe individual treatment outcomes after therapy. Based on these case reports and a review of the literature patients are usually in the 6th or 7th decade of life and present with B-symptoms or rapidly progressive adrenal insufficiency in case of bilateral involvement. The identification of bilateral adrenal masses often causes a ...

Adrenal insufficiency during physiological stress in children after kidney or liver transplantation

Pediatric Transplantation — Tue, 01 Mar 2011 00:00:00 +0100

Bilavsky E, Dagan A, Yarden‐Bilavsky H, Davidovits M, Shapiro R, Mor E, Weintrob N, Amir J, Avitzur Y. Adrenal insufficiency during physiological stress in children after kidney or liver transplantation. Pediatr Transplantation 2011. © 2011 John Wiley & Sons A/S.Abstract: The aim of this study was to assess the prevalence and risk factors of AI in pediatric recipients of kidney or liver transplantation admitted because of a physiological stress episode and to identify patients that might be at risk of adrenal crises by clinical and laboratory parameters at admission. Adrenal function was prospectively evaluated by a standard (250 μg) adrenocorticotropin test in 48 recipients. Data on clinical and laboratory parameters were collected. AI was diagnosed in 11 patients: 10/32 (3...

A case of Cushing's syndrome in pregnancy.

Endokrynologia Polska — Tue, 01 Mar 2011 00:00:00 +0100

Authors: Bednarek-Tupikowska G, Kubicka E, Sicińska-Werner T, Kazimierczak A, Winowski J, Tupikowska M, Janczyszyn M, Bolanowski M Cushing's syndrome (also known as hypercortisolemia) is rare in pregnant women due to the menstrual disturbances and infertility in women with hypercortisolism. A diagnosis of pathological hypercortisolism in pregnant women is often difficult as some symptoms of the disease may be associated with a complicated pregnancy. Hypercortisolemia leads to serious complications for mother and foetus, and is associated with premature labour and high foetal mortality. Hormonal and radiological diagnostics in pregnancy are limited. The results of hormonal measurements and dynamic tests are difficult to interpret due to the physiological changes in the hypothalamo-pitu...

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Fluticasone propionate: Adrenal insufficiency and growth failure in a child: case report

Reactions — Sun, 27 Feb 2011 18:11:29 +0100

(Source: Reactions)

Levothyroxine sodium: Adrenal insufficiency in an elderly patient: case report

Reactions — Sun, 27 Feb 2011 18:11:29 +0100

(Source: Reactions)

Chronic Actinic Dermatitis with Leonine Facies and Iatrogenic Adrenal Insufficiency Successfully Treated with Topical Tacrolimus

Karger Publishers — Thu, 24 Feb 2011 15:18:28 +0100

Case Rep Dermatol 2011;3:49–54 (DOI:10.1159/000325068) (Source: Karger Publishers)

Isolated secondary adrenal insufficiency – an underestimated consequence of asymptomatic autoimmune hypophysitis

Clinical Endocrinology — Fri, 18 Feb 2011 14:15:32 +0100

(Source: Clinical Endocrinology)

Antiphospholipid Syndrome: Endocrinologic Manifestations and Organ Involvement

Seminars in Thrombosis and Hemostasis — Wed, 09 Feb 2011 00:00:00 +0100

In this report, we present a review of the endocrinologic manifestations associated with APS by evaluating the medical literature from 1968 to 2009 using MEDLINE and these keywords: APS, antiphospholipid syndrome, antiphospholipid antibodies, anticardiolipin antibodies, lupus anticoagulant, anti β-2 glycoprotein I, pituitary, adrenal, thyroid, parathyroid, ovary, testes, diabetes mellitus, and diabetes insipidus. Adrenal insufficiency was found to be the most common endocrine manifestation associated with APS. Autoimmune thyroid disease was associated with increased titers of antiphospholipid antibodies (aPL) without any APS clinical manifestations. In addition, hypopituitarism and Sheehan syndrome are increasingly being reported in association with aPL. Data regarding the prevalence ...

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Hair Cortisol Content in Patients with Adrenal Insufficiency on Hydrocortisone Replacement Therapy

Clinical Endocrinology — Tue, 08 Feb 2011 00:00:00 +0100

Conclusions: Hair cortisol content correlates with HC dose in patients with AI. Our results suggest that some AI patients may be over‐treated and, hence, may be at risk for the adverse effects of cortisol. Measurement of HC in hair may become a useful monitoring tool for long‐term cortisol exposure in patients treated with glucocorticoids. (Source: Clinical Endocrinology)

Hormonal responses upon return of spontaneous circulation after cardiac arrest: A retrospective study

Critical Care — Mon, 07 Feb 2011 00:00:00 +0100

Conclusions: The patients with relative adrenal insufficiency and higher blood levels of ACTH and ADH upon ROSC after cardiac arrest had a poor outcome. Effectiveness of administration of cortisol and ADH to patients upon ROSC after cardiac arrest is uncertain and additional studies are needed. (Source: Critical Care)

The dynamics of post-operative plasma ACTH values following transsphenoidal surgery for Cushing’s disease

Pituitary — Sat, 05 Feb 2011 16:56:25 +0100

Abstract Rapid assessment of adrenal function is critical following transsphenoidal surgery (TSS) for Cushing’s disease (CD) in order to determine surgical efficacy. We hypothesize that there may be a role for ACTH measurement as a rapid indicator of adrenal function. Following surgery for CD, glucocorticoids were withheld and paired plasma ACTH and serum cortisol levels were measured every 6 h. Post-operative hypocortisolemia was defined as serum cortisol <2 mcg/dl or a serum cortisol <5 mcg/dl with the onset of symptoms of adrenal insufficiency within 72 h. We studied 12 subjects, all female, mean age 44.6 years (range 25–55), including 13 surgeries: nine subjects attained hypocortisolemia. Plasma ACTH levels decreased more in subjects w...

Quality of life is less impaired in adults with congenital adrenal hyperplasia because of 21‐hydroxylase deficiency than in patients with primary adrenal insufficiency

Clinical Endocrinology — Tue, 01 Feb 2011 00:00:00 +0100

Conclusions HRQoL in CAH is only mildly impaired and significantly less than in PAI patients. Differences between PAI and CAH in HRQoL suggest relevant modulating factors of HRQoL other than hormone replacement therapy itself. (Source: Clinical Endocrinology)

Mutation Spectra of the AAAS Gene in Iranian Families with Allgrove Syndrome

Archives of Medical Research — Tue, 01 Feb 2011 00:00:00 +0100

Conclusions: This is the first report of Triple-A syndrome from an Iranian population. Collectively, our study findings indicate that mutations scattered across the AAAS gene and upstream regulation elements. Various ethnic groups should develop a mutation database for their own rare genetic disorders. However, mutation databases should initially screen common mutated alleles. Our families present the typical triad of symptoms and the mutation spectrum is similar to the other population studied. Further study is required for phenotype-genotype correlation in the Iranian population. (Source: Archives of Medical Research)

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Early diagnosis of cerebral X-linked adrenoleukodystrophy in boys with Addison’s disease improves survival and neurological outcomes

European Journal of Pediatrics — Fri, 28 Jan 2011 20:31:24 +0100

In conclusion, the early diagnosis of cerebral X-ALD following the diagnosis of unexplained AI, and subsequent treatment with HCT improves both neurological outcomes and survival in boys with cerebral X-ALD. Content Type Journal ArticlePages 1-6DOI 10.1007/s00431-011-1401-1Authors Lynda E. Polgreen, Department of Pediatrics, University of Minnesota, 516 Delaware St. S.E., PWB 13-124, MMC 8404, Minneapolis, MN 55455, USASaydi Chahla, Medical School, University of Minnesota, Minneapolis, MN USAWeston Miller, Department of Pediatrics, University of Minnesota, 516 Delaware St. S.E., PWB 13-124, MMC 8404, Minneapolis, MN 55455, USASteven Rothman, Department of Pediatrics, University of Minnesota, 516 Delaware St. S.E., PWB 13-124, MMC 8404, Minneapolis, MN 55455, USAJakub Tolar, Depart...

Triamcinolone: Cushing syndrome and adrenal insufficiency following intradermal administration in a child: case report

Reactions — Mon, 24 Jan 2011 17:42:36 +0100

(Source: Reactions)

Outcome of Hand Assisted Laparoscopic Bilateral Native Nephrectomy in Transplant Recipients

The Journal of Urology — Wed, 19 Jan 2011 00:00:00 +0100

Conclusions: Simultaneous hand assisted bilateral nephrectomies are safe and reproducible. The complication rate is low and postoperative hospital stay is short compared to those in published open surgery series. Graft function was preserved in patients who underwent renal transplantation before native kidney removal. (Source: The Journal of Urology)

Tramadol-induced adrenal insufficiency

European Journal of Clinical Pharmacology — Tue, 18 Jan 2011 15:01:56 +0100

Content Type Journal ArticlePages 1-3DOI 10.1007/s00228-011-0992-9Authors Miguel Debono, Department of Diabetes and Endocrinology, Barnsley Hospital, NHS Foundation Trust, Barnsley, South Yorkshire UK S75 2PYSharon Chan, Department of Diabetes and Endocrinology, Barnsley Hospital, NHS Foundation Trust, Barnsley, South Yorkshire UK S75 2PYChristen Rolfe, Department of Diabetes and Endocrinology, Barnsley Hospital, NHS Foundation Trust, Barnsley, South Yorkshire UK S75 2PYT. Hugh Jones, Department of Diabetes and Endocrinology, Barnsley Hospital, NHS Foundation Trust, Barnsley, South Yorkshire UK S75 2PY Journal European Journal of Clinical PharmacologyOnline ISSN 1432-1041Print ISSN 0031-6970 (Source: European Journal of Clinical Pharmacology)

Sterol O-Acyltransferase 1 (SOAT1, ACAT) Is a Novel Target of Steroidogenic Factor-1 (SF-1, NR5A1, Ad4BP) in the Human Adrenal.

The Journal of Clinical Endocrinology and Metabolism — Fri, 14 Jan 2011 00:00:00 +0100

Conclusions: Our reverse discovery approach led to the identification of novel SF-1 targets and defined SOAT1 as an important factor in human adrenal steroidogenesis. SF-1-dependent up-regulation of SOAT1 may be important for maintaining readily-releasable cholesterol reserves needed for active steroidogenesis and during episodes of recurrent stress. PMID: 21239516 [PubMed - as supplied by publisher] (Source: The Journal of Clinical Endocrinology and Metabolism)

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Assessment of adrenal function in cirrhotic patients using concentration of serum‐free and salivary cortisol

Liver International — Thu, 13 Jan 2011 00:00:00 +0100

Conclusions: Serum total cortisol levels overestimated the prevalence of adrenal dysfunction in cirrhotics with end‐stage liver disease. Since serum‐free cortisol cannot be measured routinely, salivary cortisol testing could represent a useful approach but needs to be standardized. (Source: Liver International)

Auricular Cartilage Calcification and Adrenal Insufficiency

New England Journal of Medicine — Wed, 12 Jan 2011 22:00:07 +0100

New England Journal of Medicine, Volume 364, Issue 2, Page 157, January 2011. (Source: New England Journal of Medicine)

Are adults taking corticosteroids for adrenal insufficiency at risk of osteoporosis?

NeLM - Medicines Q and A — Sat, 01 Jan 2011 00:00:00 +0100

Source: North West Medicines Information Centre Area: Evidence > Medicines Q & A Evidence from observational studies indicates that some adults taking corticosteroids for adrenal insufficiency (AI) have decreased bone mineral density (BMD). Two prospective studies reported that corticosteroid replacement therapy causes a dose-dependent reduction in serum levels of osteocalcin, a surrogate marker for bone formation. The risk of bone density loss may be influenced by cumulative or daily corticosteroid dose and co-existent hormone imbalances. Accurate replacement of physiological levels of cortisol is impossible with currently available corticosteroids. There is also no objective method of monitoring the accuracy of replacement in order to avoid over- or under-treatment. The traditiona...

Pubertal Presentation in Seven Patients with Congenital Adrenal Hyperplasia due to P450 Oxidoreductase Deficiency.

The Journal of Clinical Endocrinology and Metabolism — Wed, 29 Dec 2010 00:00:00 +0100

Conclusion: Delayed and disordered puberty can be the first sign leading to a diagnosis of ORD. Appropriate testosterone production during puberty in affected boys but manifest primary hypogonadism in girls with ORD may indicate that testicular steroidogenesis is less dependent on POR than adrenal and ovarian steroidogenesis. Ovarian cysts in pubertal girls may be driven not only by high gonadotropins but possibly also by impaired CYP51A1-mediated production of meiosis-activating sterols due to mutant POR. PMID: 21190981 [PubMed - as supplied by publisher] (Source: The Journal of Clinical Endocrinology and Metabolism)

Role of DAX-1 (NR0B1) and Steroidogenic Factor-1 (NR5A1) in Human Adrenal Function.

Endocrine Development — Fri, 24 Dec 2010 21:21:13 +0100

Authors: El-Khairi R, Martinez-Aguayo A, Ferraz-de-Souza B, Lin L, Achermann JC The nuclear receptor transcription factors DAX-1 (NR0B1) and SF-1 (NR5A1) regulate many aspects of adrenal and reproductive development and function. Disruption of the genes encoding these factors can be associated with pediatric adrenal disease. DAX-1 mutations are classically associated with X-linked adrenal hypoplasia congenita, hypogonadotropic hypogonadism and impaired sper-matogenesis. However, other phenotypes are also being reported, such as isolated mineralocorti-coid insufficiency, premature sexual development, primary adrenal insufficiency in a 46, XX patient and late-onset X-linked adrenal hypoplasia congenita and/or hypogonadotropic hypogonadism. SF-1 mutations have also been associated with pr...

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Clinical and biochemical consequences of p450 oxidoreductase deficiency.

Endocrine Development — Fri, 24 Dec 2010 21:21:01 +0100

Authors: Flück CE, Pandey AV Patients with P450 oxidoreductase (POR) deficiency typically present with adrenal insufficiency, genital anomalies and bony malformations resembling the Antley-Bixler craniosynostosis syndrome. Since our first report in 2004, more than 40 POR mutations have been identified in over 65 patients. POR is the obligate electron donor to all microsomal P450 enzymes, including the steroidogenic enzymes CYP17A1, CYP21A2 and CYP19A1. POR deficiency may cause disordered sexual development manifested as genital undervirilization in 46, XY newborns as well as overvirilization in those who are 46, XX. This may be explained by impaired aromatization of fetal androgens that may cause maternal virilization and low urinary estriol levels during pregnancy. In addition, the a...

Autoimmune Addison's Disease.

Endocrine Development — Fri, 24 Dec 2010 21:20:26 +0100

Authors: Betterle C, Morlin L Primary adrenocortical insufficiency, or Addison's disease (AD), results from an adrenal cortex hypofunction/dysfunction with a deficient production of glucocorticoids, mineralocorticoids and androgens, and with high levels of both ACTH and plasma renin activity. The prevalence of AD is 110-144 cases per million population in the developed countries. Autoimmune AD is the most frequent etiological form in adult patients, accounting for about 80% of cases, followed by post-tuberculosis AD in 10-15%, the remaining 5% being cases are due to vascular, neoplastic or rare genetic forms. Congenital adrenal hyperplasia is the most frequent form of AD in children and accounts for 72% of cases, whereas autoimmune AD is seen in around 10-15% of cases. The markers of a...

Optimal glucocorticoid therapy.

Endocrine Development — Fri, 24 Dec 2010 21:20:22 +0100

Authors: Debono M, Ross RJ The rhythmic regulation of human physiology and behaviour is controlled by a central endogenous clock located in the suprachiasmatic nucleus. Most tissues have peripheral clocks that oscillate in time with this central clock. How the central time keeper controls peripheral clocks is not established, however there is evidence to suggest that the cortisol rhythm is one important secondary messenger. Loss of the endogenous cortisol rhythm is associated with sleep disturbance, depression, and metabolic abnormalities. In adrenal insufficiency, current glucocorticoid replacement regimens cannot replace the normal circadian rhythm of cortisol, and patients have an increased mortality and impaired quality of life. We propose that reproducing circadian cortisol levels...

Assessing adrenal status in patients before and immediately after coronary artery bypass graft surgery.

European Journal of Endocrinology — Thu, 23 Dec 2010 00:00:00 +0100

Conclusion: Up to one quarter of patients with a normal cortisol status preoperatively demonstrate a raised ACTH and deficient cortisol response postoperatively. Despite these responses all patients had uneventful outcomes. These data reinforce the need for caution when interpreting results of endocrine testing following major surgery or in the Intensive Care environment, and that prognostic value of these results may be of limited use. PMID: 21183553 [PubMed - as supplied by publisher] (Source: European Journal of Endocrinology)

Percutaneous Image-guided Adrenal Cryoablation: Procedural Considerations and Technical Success [Vascular and Interventional Radiology]

Radiology — Wed, 22 Dec 2010 00:00:00 +0100

Conclusion: Adrenal cryoablation is technically feasible with a high rate of local control. Patients premedicated with the -blocker phenoxybenzamine appear to have a reduced risk of hypertensive crisis. © RSNA, 2010 (Source: Radiology)

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A brugada-pattern electrocardiogram and adrenal insufficiency.

Annals of Internal Medicine — Tue, 21 Dec 2010 00:00:00 +0100

Authors: Khalil Y, Siddique SM, Hoang NT PMID: 21173421 [PubMed - in process] (Source: Annals of Internal Medicine)

Acquired long QT interval in a recurrent septic female patient with polymorphic ventricular tachycardia due to primary acute adrenal insufficiency

Clinical Research in Cardiology — Wed, 15 Dec 2010 19:11:37 +0100

Content Type Journal ArticleDOI 10.1007/s00392-010-0257-0Authors Roja Soutodeh, Department I, Cardiology, Angiology, Intensive Care, Klinikum Lippe-Detmold, Roentgenstrasse 18, 32756 Detmold, GermanyJürgen Götz, Department I, Cardiology, Angiology, Intensive Care, Klinikum Lippe-Detmold, Roentgenstrasse 18, 32756 Detmold, GermanyUlrich Tebbe, Department I, Cardiology, Angiology, Intensive Care, Klinikum Lippe-Detmold, Roentgenstrasse 18, 32756 Detmold, GermanyAlessandro Cuneo, Department I, Cardiology, Angiology, Intensive Care, Klinikum Lippe-Detmold, Roentgenstrasse 18, 32756 Detmold, Germany Journal Clinical Research in CardiologyOnline ISSN 1861-0692Print ISSN 1861-0684 (Source: Clinical Research in Cardiology)

Changes of Inflammation and Apoptosis in Adrenal Gland After Experimental Injury in Rats with Acute Necrotizing Pancreatitis

Inflammation — Wed, 15 Dec 2010 15:55:32 +0100

Abstract We hypothesize that adrenal insufficiency in acute necrotizing pancreatitis (ANP) is attributable to hemorrhagic inflammation, necrosis, and apoptosis of the adrenal cortex. Arguments to support this view are presented in the study that investigated morphological and functional changes of adrenal and the distinct roles of inflammatory mediator secretory phospholipase A2 (sPLA2) and apoptosis-related genes Bax and Bcl-2 played in acute adrenal injury in ANP. After ANP model was induced, pancreatic histology, serum amylase, sPLA2, and corticosterone were analyzed. The adrenal morphology, apoptotic cells by TUNEL assay, and ultrastructures were observed. sPLA2-IIA and Bcl-2 and Bax expressions were detected by immunohistochemistry. Histopathologic grading of adrenal wa...

Partial Defect in the Cholesterol Side-Chain Cleavage Enzyme P450scc (CYP11A1) Resembling Nonclassic Congenital Lipoid Adrenal Hyperplasia.

The Journal of Clinical Endocrinology and Metabolism — Wed, 15 Dec 2010 00:00:00 +0100

Conclusions: There is a broad clinical spectrum of P450scc deficiency. Partial loss-of-function CYP11A1 mutation can present with a hormonal phenotype indistinguishable from nonclassic lipoid CAH. PMID: 21159840 [PubMed - as supplied by publisher] (Source: The Journal of Clinical Endocrinology and Metabolism)

Etomidate and adrenal insufficiency: the controversy continues

Critical Care — Thu, 09 Dec 2010 00:00:00 +0100

No description available (Source: Critical Care)

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A young boy with diffuse hyperpigmentation and delayed puberty

European Journal of Pediatrics — Tue, 30 Nov 2010 07:00:08 +0100

Abstract An 18-year-old male patient had presented with diffuse hyperpigmentation after birth and with adrenal insufficiency syndrome since childhood. After puberty, no secondary sexual signs developed. Laboratory examination showed an extremely high concentration of serum triglycerides (9.14 mmol/L) and plasma adrenocorticotropic hormone (>275 pmol/L), however, a low concentration of plasma free cortisone (<25.1–67.6 nmol/L). Abdomen computed tomography detected atrophy of both adrenals glands. Content Type Journal ArticleDOI 10.1007/s00431-010-1357-6Authors Xian-Ling Wang, Department of Endocrinology, Chinese PLA General Hospital, 28 Fu Xing Road, Beijing, China 100853Yi-Ming Mu, Department of Endocrinology, Chinese PLA General Hospital, 28 Fu ...

Endocrine Diseases and the Liver

Clinics in Liver Disease — Sat, 27 Nov 2010 05:09:36 +0100

The objective of this review is to focus on the effect of endocrine disorders on liver. (Source: Clinics in Liver Disease)

IMAGe syndrome: Case report with a previously unreported feature and review of published literature

American Journal of Medical Genetics Part A — Thu, 25 Nov 2010 13:49:46 +0100

We report on a 7‐year‐old boy with IMAGe syndrome, who in addition to the features in the acronym also has bilateral sensorineural hearing loss which has not been reported in previously published cases of IMAGe syndrome. We discuss the clinical presentation in our patient and review the literature in this rare multisystem disorder. © 2010 Wiley‐Liss, Inc. (Source: American Journal of Medical Genetics Part A)

ACTH deficiency and PGI2 therapy in chronic thromboembolic pulmonary hypertension

International Journal of Cardiology — Fri, 19 Nov 2010 00:00:00 +0100

In conclusion, these cases suggest that CTEPH or PGI2 therapy, or both, may be associated with the development of IAD, although the mechanisms of these associations still remain undefined. ACTH deficiency should therefore be considered in the differential diagnosis when CTEPH patients, as well as patients undergoing PGI2 therapy, manifest an unexplained deterioration in symptoms. (Source: International Journal of Cardiology)