- Nivolumab, a new immunomodulatory drug, a new adverse effect; adrenal crisis. [Journal Article]
- TJTurk J Emerg Med 2017; 17(4):157-159
- Owing to the advancements in medicine, new information is obtained regarding cancer, new antineoplastic agents are developed. Frequent use of these new pharmacological agents emergency physicians to ...
Owing to the advancements in medicine, new information is obtained regarding cancer, new antineoplastic agents are developed. Frequent use of these new pharmacological agents emergency physicians to be vigilant about their side effects. We present a case of adrenal crisis in a patient with non-small cell lung cancer (NSCLC), caused by an immunomodulatory drug; nivolumab. While adverse events are related to other immunomodulatory drugs have been reported in literature, our case is the first nivolumab-related adrenal failure to be reported. A patient with lung cancer presented to the emergency room(ER) with nausea and vomiting. Hyponatremia, hyperkalemia, persistent hypoglycemia led to the diagnosis of adrenal crisis. Having direct effect on the immune system, these drugs were claimed to be highly reliable. However, there is no reliable data on the side effect profile of these agents. It should be kept in mind that life-threatening auto-immune reactions may occur.
- Acute pituitary disease in pregnancy: how to handle hypophysitis and Sheehan's syndrome? [Journal Article]
- MEMinerva Endocrinol 2018 Feb 19
- Pregnancy-related hypophysitis (PR-Hy) that typically occurs during late pregnancy and the early postpartum period is considered an autoimmune disorder. Knowledge and awareness of characteristic clin...
Pregnancy-related hypophysitis (PR-Hy) that typically occurs during late pregnancy and the early postpartum period is considered an autoimmune disorder. Knowledge and awareness of characteristic clinical symptoms and specific endocrine and neuroradiological features mostly enables a correct diagnosis and guarantees best possible management. In the presence of mild clinical symptoms, a "wait and watch" policy is recommended. Transsphenoidal surgery or glucocorticoid therapy is indicated if progressive clinical signs or severe visual symptoms are observed. Reported cases indicate that transsphenoidal surgery performed during pregnancy can reverse visual symptoms and has a minimal risk to the health of the fetus and mother. Glucocorticoid therapy has a high initial response rate but recurrence can occur after discontinuation. In late-pregnancy hypophysitis, the options of delaying therapy until the child is born or, if symptoms are severe, of inducing delivery prior to therapy have to be considered. Sheehan's syndrome (SS) is characterized by irreversible, partial or complete hypopituitarism due to pituitary infarction. The predominating causative event is massive peripartum hemorrhage (PPH). A considerable diagnostic delay is mostly observed. PPH and the typical postpartum findings of agalactia and amenorrhea should alert for the diagnosis of SS and prompt endocrine evaluation. Timely hormone replacement therapy is paramount to reduce morbidity and mortality and improve quality of life of afflicted women. Adrenal failure is a major endocrinological risk both of hypophysitis and of Sheehan syndrome. Early recognition and treatment is paramount to avoid life-threatening adrenal crisis.
- Incidence and Characteristics of Adrenal Crisis in Children Younger than 7 Years with 21-Hydroxylase Deficiency: A Nationwide Survey in Japan. [Journal Article]
- HRHorm Res Paediatr 2018 Feb 16
- CONCLUSIONS: Adrenal crisis is not rare and can be accompanied by disastrous hypoglycemia in children with 21-OHD.
- Impact of opioid therapy on gonadal hormones: focus on buprenorphine. [Review]
- HMHorm Mol Biol Clin Investig 2018 Feb 17
- Objective The USA is in the midst of an opioid crisis. Understanding the impact of opioids and commonly used treatments for opioid dependence is essential for clinicians and researchers in order to e...
Objective The USA is in the midst of an opioid crisis. Understanding the impact of opioids and commonly used treatments for opioid dependence is essential for clinicians and researchers in order to educate and treat the nation's growing population with opioid use disorders. As a relatively new treatment for opioid dependence, buprenorphine is gaining popularity to the extent of becoming not only a preferred approach to the maintenance of opiate addiction, but also an option for chronic pain management. The purpose of this report is to review the available evidence on the endocrine effects of buprenorphine, particularly as it relates to the hypothalamic-pituitary-gonadal (HPG) axis, which is controversial and not fully defined. Method We conducted a Pubmed search (2000-2017) for human studies in the English language for articles that were available as full length regarding buprenorphine, endocrinopathy, hypogonadism, bone density, opioids. Case reports were also reviewed, although prospective studies and randomized controlled trials received more weight. Results Opioid induced hypogonadism is well established. Most studies report that buprenorphine being a partial agonist/antagonist may not be impacting the pituitary trophic hormones as much. There are reports of sexual dysfunction in subjects maintained on buprenorphine, some without hormonal correlation. Thus with the understanding that pertinent clinical studies are limited in number, varied in methodology, mostly cross sectional, predominantly in men and small number of participants, more research in this area is warranted. Conclusion Based on a comprehensive review of the available literature, we conclude that despite its increasing popularity, buprenorphine has not been adequately studied in respect to its long-term effects on the hypothalamic-pituitary-adrenal (HPA) axis. There is a great need for longitudinal systematic trials to define the potential buprenorphine-induced endocrine consequences.
- Retroperitoneal laparoscopic adrenalectomy with transient renal artery occlusion for large adrenal tumors (≥8 cm). [Journal Article]
- JSJ Surg Oncol 2018 Feb 15
- CONCLUSIONS: RLA with transient renal artery occlusion is a feasible, effective, and safe treatment for large adrenal tumors (≥8 cm).
- Primary adrenal insufficiency due to hereditary apolipoprotein AI amyloidosis: endocrine involvement beyond hypogonadism. [Journal Article]
- AAmyloid 2018 Feb 15; :1-4
- Several mutations in the gene encoding apolipoprotein AI (apoAI) have been described as a cause of familial amyloidosis. Individuals with apoAI-derived (AApoAI) amyloidosis frequently manifest with l...
Several mutations in the gene encoding apolipoprotein AI (apoAI) have been described as a cause of familial amyloidosis. Individuals with apoAI-derived (AApoAI) amyloidosis frequently manifest with liver, kidney, laryngeal, skin and myocardial involvement. Although primary hypogonadism (PH) is considered almost pathognomonic of this disease, until now, primary adrenal insufficiency (PAI) has not been described as a common clinical feature. Here, we report the first kindred with AApoAI amyloidosis in which PAI is well-documented. All family members with the Leu60_Phe71delins60Val_61Thr heterozygous mutation who were regularly followed-up at our centre were considered. Nineteen individuals had the confirmed APOA1 deletion/insertion mutation, with detailed medical records available in 11 cases. Of these, 6 had PAI and 3 (all males) had PH. Among them, one 47-year-old man, not previously diagnosed with PAI, developed adrenal crisis after liver transplantation, precipitated by an opportunistic infection. Transplantation due to organ failure, which necessitates use of immunosuppressive medication such as corticosteroids, is frequently required during the course of hereditary amyloidosis. Consequently, PAI can remain masked, being discovered only when an adrenal crisis develops. Therefore, according to the present evidence, patients with AApoAI amyloidosis should be submitted to regular testing of corticotrophin and cortisol levels in order to avoid delaying corticosteroid replacement.
- [Steroid 21-hydroxylase deficiency, the most frequent cause of congenital adrenal hyperplasia]. [Journal Article]
- OHOrv Hetil 2018; 159(7):269-277
- Congenital adrenal hyperplasia is a group of genetic diseases due to the disablement of 7 genes; one of them is steroid 21-hydroxylase deficiency. The genes of congenital adrenal hyperplasia encode e...
Congenital adrenal hyperplasia is a group of genetic diseases due to the disablement of 7 genes; one of them is steroid 21-hydroxylase deficiency. The genes of congenital adrenal hyperplasia encode enzymes taking part in the steroidogenesis of adrenal gland. Steroid 21-hydroxylase deficiency is an autosomal recessive disorder caused by mutations of the steroid 21-hydroxylase gene. The mutations of steroid 21-hydroxylase gene cause 95% of the congenital adrenal hyperplasia cases. Although the non-classic steroid 21-hydroxylase deficiency with mild symptoms is seldom diagnosed, the classic steroid 21-hydroxylase deficiency may lead to life-threatening salt-wasting and adrenal crises due to the insufficient aldosterone and cortisol serum levels. The classic type requires life-long steroid replacement which may result in cushingoid side effects, and typical comorbidities may be also developed. The patients' quality of life is decreased, and their mortality is much higher than that of the population without steroid 21-hydroxylase deficiency. The diagnosis, consequences and the patients' life-long clinical care require a multidisciplinary approach: the specialists in pediatrics, internal medicine, endocrinology, laboratory medicine, genetic diagnostics, surgery, obstetrics-gynecology and psychology need to work together. Orv Hetil. 2018; 159(7): 269-277.
- Evaluation of the frequency of adrenal crises and preventive measures in patients with primary and secondary adrenal insufficiency in Switzerland. [Journal Article]
- SMSwiss Med Wkly 2018 Jan 29; 148:w14586
- CONCLUSIONS: The incidence of adrenal crisis in Switzerland is lower than described in recent European studies. Although nearly all of the patients carry their emergency cards with them, emergency treatment is available in only about half of the patients. There is a mismatch between subjective and objective knowledge of the disease and the education of patients with adrenal insufficiency needs to be improved.
- GeneReviews® [BOOK]
- BOOKUniversity of Washington, Seattle: Seattle (WA)
- NR0B1- related adrenal hypoplasia congenita includes both X-linked adrenal hypoplasia congenita (X-linked AHC) and Xp21 deletion (previously called complex glycerol kinase deficiency)...
NR0B1- related adrenal hypoplasia congenita includes both X-linked adrenal hypoplasia congenita (X-linked AHC) and Xp21 deletion (previously called complex glycerol kinase deficiency). X-linked AHC is characterized by primary adrenal insufficiency and/or hypogonadotropic hypogonadism (HH). Adrenal insufficiency is acute infantile onset (average age 3 weeks) in approximately 60% of affected males and childhood onset (ages 1-9 years) in approximately 40%. HH typically manifests in a male with adrenal insufficiency as delayed puberty (i.e., onset age >14 years) and less commonly as arrested puberty at about Tanner Stage 3. Rarely, X-linked AHC manifests initially in early adulthood as delayed-onset adrenal insufficiency, partial HH, and/or infertility. Heterozygous females very occasionally have manifestations of adrenal insufficiency or hypogonadotropic hypogonadism. Xp21 deletion includes deletion ofNR0B1(causing X-linked AHC) andGK(causing glycerol kinase deficiency), and in some cases deletion ofDMD(causing Duchenne muscular dystrophy). Developmental delay has been reported in males with Xp21 deletion when the deletion extends proximally to includeDMDor when larger deletions extend distally to includeIL1RAPL1andDMD.
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- Effectiveness and Safety of CT-guided Percutaneous Radiofrequency Ablation of Adrenal Metastases. [Journal Article]
- BJBr J Radiol 2018 Jan 19; :20170607
- CONCLUSIONS: The current study demonstrated that radiofrequency ablation is a relatively safe and effective treatment for controlling adrenal metastases, especially for patients with tumor size < 3cm. Advances in knowledge: Surgical resection of the adrenal metastases was advocated as one of the treatment options for patients. The present study showed that radiofrequency ablation is a relatively safe and effective treatment for controlling adrenal metastases.